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0000009_1
Ewing Sarcoma
6
0000009_1-6
treatment
What are the treatments for Ewing Sarcoma ?
Key Points - There are different types of treatment for children with Ewing sarcoma. - Children with Ewing sarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children. - Treatment for Ewing sarcoma may cause side effects. - Five types of standard treatment are used: - Chemotherapy - Radiation therapy - Surgery - Targeted therapy - High-dose chemotherapy with stem cell rescue - New types of treatment are being tested in clinical trials. - Chimeric antigen receptor (CAR) T-cell therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for children with Ewing sarcoma. Different types of treatments are available for children with Ewing sarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with Ewing sarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with Ewing sarcoma and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Surgical oncologist or orthopedic oncologist. - Radiation oncologist. - Pediatric nurse specialist. - Social worker. - Rehabilitation specialist. - Psychologist. Treatment for Ewing sarcoma may cause side effects. For information about side effects that begin during treatment for cancer, see our Side Effects page. Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following: - Physical problems. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer). Patients treated for Ewing sarcoma have an increased risk of acute myeloid leukemia and myelodysplastic syndrome. There is also an increased risk of sarcoma in the area treated with radiation therapy. Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) Five types of standard treatment are used: Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. Systemic chemotherapy is part of the treatment for all patients with Ewing tumors. It is often the first treatment given and lasts for about 6 to 12 months. Chemotherapy is often given to shrink the tumor before surgery or radiation therapy and to kill any tumor cells that may have spread to other parts of the body. See Drugs Approved for Soft Tissue Sarcoma for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. External radiation therapy is used to treat Ewing sarcoma. Radiation therapy is used when the tumor cannot be removed by surgery or when surgery to remove the tumor will affect important body functions or the way the child will look. It may be used to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. It may also be used to treat any tumor that remains after surgery and tumors that have spread to other parts of the body. Surgery Surgery is usually done to remove cancer that is left after chemotherapy or radiation therapy. When possible, the whole tumor is removed by surgery. Tissue and bone that are removed may be replaced with a graft, which uses tissue and bone taken from another part of the patient's body or a donor. Sometimes an implant, such as artificial bone, is used. Even if the doctor removes all of the cancer that can be seen at the time of the operation, chemotherapy or radiation therapy may be given after surgery to kill any cancer cells that are left. Chemotherapy or radiation therapy given after surgery to lower the risk that the cancer will come back is called adjuvant therapy. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is a type of targeted therapy used in the treatment of recurrent Ewing sarcoma. It is being studied for the treatment of metastatic Ewing sarcoma. Monoclonal antibodies are made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. New types of targeted therapy are being studied. - Kinase inhibitor therapy is another type of targeted therapy. Kinase inhibitors are drugs that block a protein needed for cancer cells to divide. They are being studied in the treatment of recurrent Ewing sarcoma. - PARP inhibitor therapy is another type of targeted therapy. PARP inhibitors are drugs that block DNA repair and may cause cancer cells to die. They are being studied in the treatment of recurrent Ewing sarcoma. High-dose chemotherapy with stem cell rescue High-dose chemotherapy with stem cell rescue is a way of giving high doses of chemotherapy to treat Ewing sarcoma and then replacing blood -forming cells destroyed by cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Chemotherapy with stem cell rescue is used to treat recurrent Ewing sarcoma. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chimeric antigen receptor (CAR) T-cell therapy CAR T-cell therapy is a type of immunotherapy that changes the patient's T cells (a type of immune system cell) so they will attack certain proteins on the surface of cancer cells. T cells are taken from the patient and special receptors are added to their surface in the laboratory. The changed cells are called chimeric antigen receptor (CAR) T cells. The CAR T cells are grown in the laboratory and given to the patient by infusion. The CAR T cells multiply in the patient's blood and attack cancer cells. CAR T-cell therapy is being studied in the treatment of Ewing sarcoma that has recurred. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Ewing Sarcoma Localized Ewing Sarcoma Standard treatments for localized Ewing sarcoma include: - Chemotherapy. - Surgery and/or radiation therapy. These treatments and the order they are given depend on the following: - Where in the body the tumor started. - How large the tumor is when the cancer is diagnosed. - Whether the tumor was completely removed by surgery. - The child's age and general health. - Whether the treatment will affect important body functions or the way the child will look. Treatments being studied for localized Ewing sarcoma include: - High-dose chemotherapy with stem cell rescue. Check the list of NCI-supported cancer clinical trials that are now accepting patients with localized Ewing sarcoma/peripheral primitive neuroectodermal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Metastatic Ewing Sarcoma Standard treatments for metastatic Ewing sarcoma include: - Chemotherapy. - Surgery. - Radiation therapy. These treatments and the order they are given depend on the following: - Where in the body the tumor started. - Where the tumor has spread. - How large the tumor is. - Whether the treatment will affect important body functions or the way the child will look. - The child's age and general health. Treatments being studied for metastatic Ewing sarcoma include the following: - Combination chemotherapy with or without targeted therapy. Radiation therapy is given to areas of bone where cancer has spread. - High-dose chemotherapy with stem cell rescue. Check the list of NCI-supported cancer clinical trials that are now accepting patients with metastatic Ewing sarcoma/peripheral primitive neuroectodermal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Recurrent Ewing Sarcoma There is no standard treatment for recurrent Ewing sarcoma but treatment options may include the following: - Combination chemotherapy. - Radiation therapy to bone tumors, as palliative therapy to relieve symptoms and improve the quality of life. - Radiation therapy that may be followed by surgery to remove tumors that have spread to the lungs. - High-dose chemotherapy with stem cell rescue. - Targeted therapy with a monoclonal antibody. These treatments and the order they are given depend on the following: - Where in the body the tumor came back. - The initial treatment given. Treatment options being studied for recurrent Ewing sarcoma include the following: - Targeted therapy with a monoclonal antibody. - Chimeric antigen receptor (CAR) T-cell therapy. - Targeted therapy with a PARP inhibitor and chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent Ewing sarcoma/peripheral primitive neuroectodermal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
0000009_1
Ewing Sarcoma
7
0000009_1-7
research
what research (or clinical trials) is being done for Ewing Sarcoma ?
New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chimeric antigen receptor (CAR) T-cell therapy CAR T-cell therapy is a type of immunotherapy that changes the patient's T cells (a type of immune system cell) so they will attack certain proteins on the surface of cancer cells. T cells are taken from the patient and special receptors are added to their surface in the laboratory. The changed cells are called chimeric antigen receptor (CAR) T cells. The CAR T cells are grown in the laboratory and given to the patient by infusion. The CAR T cells multiply in the patient's blood and attack cancer cells. CAR T-cell therapy is being studied in the treatment of Ewing sarcoma that has recurred. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
0000033_1
Parathyroid Cancer
1
0000033_1-1
information
What is (are) Parathyroid Cancer ?
Key Points - Parathyroid cancer is a rare disease in which malignant (cancer) cells form in the tissues of a parathyroid gland. - Having certain inherited disorders can increase the risk of developing parathyroid cancer. - Signs and symptoms of parathyroid cancer include weakness, feeling tired, and a lump in the neck. - Tests that examine the neck and blood are used to detect (find) and diagnose parathyroid cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Parathyroid cancer is a rare disease in which malignant (cancer) cells form in the tissues of a parathyroid gland. The parathyroid glands are four pea-sized organs found in the neck near the thyroid gland. The parathyroid glands make parathyroid hormone (PTH or parathormone). PTH helps the body use and store calcium to keep the calcium in the blood at normal levels. A parathyroid gland may become overactive and make too much PTH, a condition called hyperparathyroidism. Hyperparathyroidism can occur when a benign tumor (noncancer), called an adenoma, forms on one of the parathyroid glands, and causes it to grow and become overactive. Sometimes hyperparathyroidism can be caused by parathyroid cancer, but this is very rare. The extra PTH causes: - The calcium stored in the bones to move into the blood. - The intestines to absorb more calcium from the food we eat. This condition is called hypercalcemia (too much calcium in the blood). The hypercalcemia caused by hyperparathyroidism is more serious and life-threatening than parathyroid cancer itself and treating hypercalcemia is as important as treating the cancer.
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Parathyroid Cancer
2
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susceptibility
Who is at risk for Parathyroid Cancer? ?
Having certain inherited disorders can increase the risk of developing parathyroid cancer. Anything that increases the chance of getting a disease is called a risk factor. Risk factors for parathyroid cancer include the following rare disorders that are inherited (passed down from parent to child): - Familial isolated hyperparathyroidism (FIHP). - Multiple endocrine neoplasia type 1 (MEN1) syndrome. Treatment with radiation therapy may increase the risk of developing a parathyroid adenoma.
0000033_1
Parathyroid Cancer
3
0000033_1-3
symptoms
What are the symptoms of Parathyroid Cancer ?
Signs and symptoms of parathyroid cancer include weakness, feeling tired, and a lump in the neck. Most parathyroid cancer signs and symptoms are caused by the hypercalcemia that develops. Signs and symptoms of hypercalcemia include the following: - Weakness. - Feeling very tired. - Nausea and vomiting. - Loss of appetite. - Weight loss for no known reason. - Being much more thirsty than usual. - Urinating much more than usual. - Constipation. - Trouble thinking clearly. Other signs and symptoms of parathyroid cancer include the following: - Pain in the abdomen, side, or back that doesn't go away. - Pain in the bones. - A broken bone. - A lump in the neck. - Change in voice such as hoarseness. - Trouble swallowing. Other conditions may cause the same signs and symptoms as parathyroid cancer. Check with your doctor if you have any of these problems.
0000033_1
Parathyroid Cancer
4
0000033_1-4
exams and tests
How to diagnose Parathyroid Cancer ?
Tests that examine the neck and blood are used to detect (find) and diagnose parathyroid cancer. Once blood tests are done and hyperparathyroidism is diagnosed, imaging tests may be done to help find which of the parathyroid glands is overactive. Sometimes the parathyroid glands are hard to find and imaging tests are done to find exactly where they are. Parathyroid cancer may be hard to diagnose because the cells of a benign parathyroid adenoma and a malignant parathyroid cancer look alike. The patient's symptoms, blood levels of calcium and parathyroid hormone, and characteristics of the tumor are also used to make a diagnosis. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. To diagnose parathyroid cancer, the sample of blood is checked for its calcium level. - Parathyroid hormone test: A procedure in which a blood sample is checked to measure the amount of parathyroid hormone released into the blood by the parathyroid glands. A higher than normal amount of parathyroid hormone can be a sign of disease. - Sestamibi scan : A type of radionuclide scan used to find an overactive parathyroid gland. A very small amount of a radioactive substance called technetium 99 is injected into a vein and travels through the bloodstream to the parathyroid gland. The radioactive substance will collect in the overactive gland and show up brightly on a special camera that detects radioactivity. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - SPECT scan (single photon emission computed tomography scan): A procedure that uses a special camera linked to a computer to make a 3-dimensional (3-D) picture. A very small amount of a radioactive substance is injected into a vein. As the substance travels through the blood, the camera rotates around the neck and takes pictures. Blood flow and metabolism are higher than normal in areas where cancer cells are growing. These areas will show up brighter in the picture. This procedure may be done just before or after a CT scan. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. - Angiogram : A procedure to look at blood vessels and the flow of blood. A contrast dye is injected into the blood vessel. As the contrast dye moves through the blood vessel, x-rays are taken to see if there are any blockages. - Venous sampling : A procedure in which a sample of blood is taken from specific veins and checked to measure the amounts of certain substances released into the blood by nearby organs and tissues. If imaging tests do not show which parathyroid gland is overactive, blood samples may be taken from veins near each parathyroid gland to find which one is making too much PTH.
0000033_1
Parathyroid Cancer
5
0000033_1-5
outlook
What is the outlook for Parathyroid Cancer ?
Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - Whether the calcium level in the blood can be controlled. - The stage of the cancer. - Whether the tumor and the capsule around the tumor can be completely removed by surgery. - The patient's general health.
0000033_1
Parathyroid Cancer
6
0000033_1-6
stages
What are the stages of Parathyroid Cancer ?
Key Points - After parathyroid cancer has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - There is no standard staging process for parathyroid cancer. After parathyroid cancer has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. The process used to find out if cancer has spread to other parts of the body is called staging. The following imaging tests may be used to determine if cancer has spread to other parts of the body such as the lungs, liver, bone, heart, pancreas, or lymph nodes: - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if parathyroid cancer spreads to the lung, the cancer cells in the lung are actually parathyroid cancer cells. The disease is metastatic parathyroid cancer, not lung cancer. There is no standard staging process for parathyroid cancer. Parathyroid cancer is described as either localized or metastatic: - Localized parathyroid cancer is found in a parathyroid gland and may have spread to nearby tissues. - Metastatic parathyroid cancer has spread to other parts of the body, such as the lungs, liver, bone, sac around the heart, pancreas, or lymph nodes.
0000033_1
Parathyroid Cancer
7
0000033_1-7
treatment
What are the treatments for Parathyroid Cancer ?
Key Points - There are different types of treatment for patients with parathyroid cancer. - Treatment includes control of hypercalcemia (too much calcium in the blood) in patients who have an overactive parathyroid gland. - Four types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Supportive care - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with parathyroid cancer. Different types of treatment are available for patients with parathyroid cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Treatment includes control of hypercalcemia (too much calcium in the blood) in patients who have an overactive parathyroid gland. In order to reduce the amount of parathyroid hormone that is being made and control the level of calcium in the blood, as much of the tumor as possible is removed in surgery. For patients who cannot have surgery, medication may be used. Four types of standard treatment are used: Surgery Surgery (removing the cancer in an operation) is the most common treatment for parathyroid cancer that is in the parathyroid glands or has spread to other parts of the body. Because parathyroid cancer grows very slowly, cancer that has spread to other parts of the body may be removed by surgery in order to cure the patient or control the effects of the disease for a long time. Before surgery, treatment is given to control hypercalcemia. The following surgical procedures may be used: - En bloc resection: Surgery to remove the entire parathyroid gland and the capsule around it. Sometimes lymph nodes, half of the thyroid gland on the same side of the body as the cancer, and muscles, tissues, and a nerve in the neck are also removed. - Tumor debulking: A surgical procedure in which as much of the tumor as possible is removed. Some tumors cannot be completely removed. - Metastasectomy: Surgery to remove any cancer that has spread to distant organs such as the lung. Surgery for parathyroid cancer sometimes damages nerves of the vocal cords. There are treatments to help with speech problems caused by this nerve damage. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat parathyroid cancer. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Supportive care Supportive care is given to lessen the problems caused by the disease or its treatment. Supportive care for hypercalcemia caused by parathyroid cancer may include the following: - Intravenous (IV) fluids. - Drugs that increase how much urine the body makes. - Drugs that stop the body from absorbing calcium from the food we eat. - Drugs that stop the parathyroid gland from making parathyroid hormone. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Parathyroid cancer often recurs. Patients should have regular check-ups for the rest of their lives, to find and treat recurrences early. Treatment Options for Parathyroid Cancer Localized Parathyroid Cancer Treatment of localized parathyroid cancer may include the following: - Surgery (en bloc resection). - Surgery followed by radiation therapy. - Radiation therapy. - Supportive care to treat hypercalcemia (too much calcium in the blood). Check the list of NCI-supported cancer clinical trials that are now accepting patients with localized parathyroid cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Metastatic Parathyroid Cancer Treatment of metastatic parathyroid cancer may include the following: - Surgery (metastasectomy) to remove cancer from the places where it has spread. - Surgery followed by radiation therapy. - Radiation therapy. - Chemotherapy. - Supportive care to treat hypercalcemia (too much calcium in the blood). Check the list of NCI-supported cancer clinical trials that are now accepting patients with metastatic parathyroid cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Parathyroid Cancer Treatment of recurrent parathyroid cancer may include the following: - Surgery (metastasectomy) to remove cancer from the places where it has recurred. - Surgery (tumor debulking). - Surgery followed by radiation therapy. - Radiation therapy. - Chemotherapy. - Supportive care to treat hypercalcemia (too much calcium in the blood). Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent parathyroid cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
0000033_1
Parathyroid Cancer
8
0000033_1-8
research
what research (or clinical trials) is being done for Parathyroid Cancer ?
New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
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Adult Hodgkin Lymphoma
1
0000004_1-1
information
What is (are) Adult Hodgkin Lymphoma ?
Key Points - Adult Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system. - There are two main types of Hodgkin lymphoma: classical and nodular lymphocyte-predominant. - Age, gender, and Epstein-Barr infection can affect the risk of adult Hodgkin lymphoma. - Signs of adult Hodgkin lymphoma include swollen lymph nodes, fever, night sweats, and weight loss. - Tests that examine the lymph nodes are used to detect (find) and diagnose adult Hodgkin lymphoma. - Certain factors affect prognosis (chance of recovery) and treatment options. Adult Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system. Adult Hodgkin lymphoma is a type of cancer that develops in the lymph system, part of the body's immune system. The immune system protects the body from foreign substances, infection, and diseases. The lymph system is made up of the following: - Lymph: Colorless, watery fluid that carries white blood cells called lymphocytes through the lymph system. Lymphocytes protect the body against infections and the growth of tumors. - Lymph vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream. - Lymph nodes: Small, bean-shaped structures that filter lymph and store white blood cells that help fight infection and disease. Lymph nodes are located along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the neck, underarm, abdomen, pelvis, and groin. - Spleen: An organ that makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. It is located on the left side of the abdomen near the stomach. - Thymus: An organ in which lymphocytes grow and multiply. The thymus is in the chest behind the breastbone. - Tonsils: Two small masses of lymph tissue at the back of the throat. The tonsils make lymphocytes. - Bone marrow: The soft, spongy tissue in the center of large bones. Bone marrow makes white blood cells, red blood cells, and platelets. Lymph tissue is also found in other parts of the body such as the stomach, thyroid gland, brain, and skin. Cancer can spread to the liver and lungs. Lymphomas are divided into two general types: Hodgkin lymphoma and non-Hodgkin lymphoma. This summary is about the treatment of adult Hodgkin lymphoma. (See the PDQ summary on Adult Non-Hodgkin Lymphoma Treatment for more information.) Hodgkin lymphoma can occur in both adults and children. Treatment for adults is different than treatment for children. Hodgkin lymphoma may also occur in patients who have acquired immunodeficiency syndrome (AIDS); these patients require special treatment. See the following PDQ summaries for more information: - Childhood Hodgkin Lymphoma Treatment - AIDS-Related Lymphoma Treatment Hodgkin lymphoma in pregnant women is the same as the disease in nonpregnant women of childbearing age. However, treatment is different for pregnant women. This summary includes information about treating Hodgkin lymphoma during pregnancy. There are two main types of Hodgkin lymphoma: classical and nodular lymphocyte-predominant. Most Hodgkin lymphomas are the classical type. The classical type is broken down into the following four subtypes: - Nodular sclerosing Hodgkin lymphoma. - Mixed cellularity Hodgkin lymphoma. - Lymphocyte depletion Hodgkin lymphoma. - Lymphocyte-rich classical Hodgkin lymphoma.
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Adult Hodgkin Lymphoma
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susceptibility
Who is at risk for Adult Hodgkin Lymphoma? ?
Age, gender, and Epstein-Barr infection can affect the risk of adult Hodgkin lymphoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for adult Hodgkin lymphoma include the following: - Being in young or late adulthood. - Being male. - Being infected with the Epstein-Barr virus. - Having a first-degree relative (parent, brother, or sister) with Hodgkin lymphoma. Pregnancy is not a risk factor for Hodgkin lymphoma.
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Adult Hodgkin Lymphoma
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symptoms
What are the symptoms of Adult Hodgkin Lymphoma ?
Signs of adult Hodgkin lymphoma include swollen lymph nodes, fever, night sweats, and weight loss. These and other signs and symptoms may be caused by adult Hodgkin lymphoma or by other conditions. Check with your doctor if any of the following do not go away: - Painless, swollen lymph nodes in the neck, underarm, or groin. - Fever for no known reason. - Drenching night sweats. - Weight loss for no known reason. - Itchy skin. - Feeling very tired.
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Adult Hodgkin Lymphoma
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exams and tests
How to diagnose Adult Hodgkin Lymphoma ?
Tests that examine the lymph nodes are used to detect (find) and diagnose adult Hodgkin lymphoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's past illnesses and treatments will also be taken. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the sample made up of red blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Sedimentation rate : A procedure in which a sample of blood is drawn and checked for the rate at which the red blood cells settle to the bottom of the test tube. The sedimentation rate is a measure of how much inflammation is in the body. A higher than normal sedimentation rate may be a sign of lymphoma or another condition. Also called erythrocyte sedimentation rate, sed rate, or ESR. - Lymph node biopsy : The removal of all or part of a lymph node. One of the following types of biopsies may be done: - Excisional biopsy : The removal of an entire lymph node. - Incisional biopsy : The removal of part of a lymph node. - Core biopsy : The removal of part of a lymph node using a wide needle. A pathologist views the tissue under a microscope to look for cancer cells, especially Reed-Sternberg cells. Reed-Sternberg cells are common in classical Hodgkin lymphoma. The following test may be done on tissue that was removed: - Immunophenotyping : A laboratory test used to identify cells, based on the types of antigens or markers on the surface of the cell. This test is used to diagnose the specific type of lymphoma by comparing the cancer cells to normal cells of the immune system.
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Adult Hodgkin Lymphoma
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outlook
What is the outlook for Adult Hodgkin Lymphoma ?
Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The patient's signs and symptoms. - The stage of the cancer. - The type of Hodgkin lymphoma. - Blood test results. - The patient's age, gender, and general health. - Whether the cancer is recurrent or progressive. For Hodgkin lymphoma during pregnancy, treatment options also depend on: - The wishes of the patient. - The age of the fetus. Adult Hodgkin lymphoma can usually be cured if found and treated early.
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Adult Hodgkin Lymphoma
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research
what research (or clinical trials) is being done for Adult Hodgkin Lymphoma ?
New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemotherapy and radiation therapy with stem cell transplant High-dose chemotherapy and radiation therapy with stem cell transplant is a way of giving high doses of chemotherapy and radiation therapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After therapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. The use of lower-dose chemotherapy and radiation therapy with stem cell transplant is also being studied. Monoclonal antibody therapy Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
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Adult Hodgkin Lymphoma
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stages
What are the stages of Adult Hodgkin Lymphoma ?
Key Points - After adult Hodgkin lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body. - There are three ways that cancer spreads in the body. - Stages of adult Hodgkin lymphoma may include A, B, E, and S. - The following stages are used for adult Hodgkin lymphoma: - Stage I - Stage II - Stage III - Stage IV - Adult Hodgkin lymphoma may be grouped for treatment as follows: - Early Favorable - Early Unfavorable - Advanced After adult Hodgkin lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body. The process used to find out if cancer has spread within the lymph system or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. For adult Hodgkin lymphoma, CT scans of the neck, chest, abdomen, and pelvis are taken. - PET-CT scan : A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself. A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer. For pregnant women with Hodgkin lymphoma, staging tests that protect the fetus from the harms of radiation are used. These include: - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Stages of adult Hodgkin lymphoma may include A, B, E, and S. Adult Hodgkin lymphoma may be described as follows: - A: The patient does not have B symptoms (fever, weight loss, or night sweats). - B: The patient has B symptoms. - E: Cancer is found in an organ or tissue that is not part of the lymph system but which may be next to an involved area of the lymph system. - S: Cancer is found in the spleen. The following stages are used for adult Hodgkin lymphoma: Stage I Stage I is divided into stage I and stage IE. - Stage I: Cancer is found in one of the following places in the lymph system: - One or more lymph nodes in one lymph node group. - Waldeyer's ring. - Thymus. - Spleen. - Stage IE: Cancer is found outside the lymph system in one organ or area. Stage II Stage II is divided into stage II and stage IIE. - Stage II: Cancer is found in two or more lymph node groups either above or below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen). - Stage IIE: Cancer is found in one or more lymph node groups either above or below the diaphragm and outside the lymph nodes in a nearby organ or area. Stage III Stage III is divided into stage III, stage IIIE, stage IIIS, and stage IIIE,S. - Stage III: Cancer is found in lymph node groups above and below the diaphragm (the thin muscle below the lungs that helps breathing and separates the chest from the abdomen). - Stage IIIE: Cancer is found in lymph node groups above and below the diaphragm and outside the lymph nodes in a nearby organ or area. - Stage IIIS: Cancer is found in lymph node groups above and below the diaphragm, and in the spleen. - Stage IIIE,S: Cancer is found in lymph node groups above and below the diaphragm, outside the lymph nodes in a nearby organ or area, and in the spleen. Stage IV In stage IV, the cancer: - is found outside the lymph nodes throughout one or more organs, and may be in lymph nodes near those organs; or - is found outside the lymph nodes in one organ and has spread to areas far away from that organ; or - is found in the lung, liver, bone marrow, or cerebrospinal fluid (CSF). The cancer has not spread to the lung, liver, bone marrow, or CSF from nearby areas. Adult Hodgkin lymphoma may be grouped for treatment as follows: Early Favorable Early favorable adult Hodgkin lymphoma is stage I or stage II, without risk factors. Early Unfavorable Early unfavorable adult Hodgkin lymphoma is stage I or stage II with one or more of the following risk factors: - A tumor in the chest that is larger than 1/3 of the width of the chest or at least 10 centimeters. - Cancer in an organ other than the lymph nodes. - A high sedimentation rate (in a sample of blood, the red blood cells settle to the bottom of the test tube more quickly than normal). - Three or more lymph nodes with cancer. - Symptoms such as fever, weight loss, or night sweats. Advanced Advanced Hodgkin lymphoma includes some or all of the following risk factors: - Being male. - Being aged 45 years or older. - Having stage IV disease. - Having a low blood albumin (protein) level (below 4). - Having a low hemoglobin level (below 10.5). - Having a high white blood cell count (15,000 or higher). - Having a low lymphocyte count (below 600 or less than 8% of the white blood cell count).
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Adult Hodgkin Lymphoma
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treatment
What are the treatments for Adult Hodgkin Lymphoma ?
Key Points - There are different types of treatment for patients with adult Hodgkin lymphoma. - Patients with Hodgkin lymphoma should have their treatment planned by a team of health care providers with expertise in treating lymphomas. - Patients may develop late effects that appear months or years after their treatment for Hodgkin lymphoma. - Three types of standard treatment are used: - Chemotherapy - Radiation therapy - Surgery - For pregnant patients with Hodgkin lymphoma, treatment options also include: - Watchful waiting - Steroid therapy - New types of treatment are being tested in clinical trials. - Chemotherapy and radiation therapy with stem cell transplant - Monoclonal antibody therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with adult Hodgkin lymphoma. Different types of treatment are available for patients with adult Hodgkin lymphoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. For pregnant women with Hodgkin lymphoma, treatment is carefully chosen to protect the fetus. Treatment decisions are based on the mothers wishes, the stage of the Hodgkin lymphoma, and the age of the fetus. The treatment plan may change as the signs and symptoms, cancer, and pregnancy change. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Patients with Hodgkin lymphoma should have their treatment planned by a team of health care providers with expertise in treating lymphomas. Treatment will be overseen by a medical oncologist, a doctor who specializes in treating cancer. The medical oncologist may refer you to other health care providers who have experience and expertise in treating adult Hodgkin lymphoma and who specialize in certain areas of medicine. These may include the following specialists: - Neurosurgeon. - Neurologist. - Rehabilitation specialist. - Radiation oncologist. - Endocrinologist. - Hematologist. - Other oncology specialists. Patients may develop late effects that appear months or years after their treatment for Hodgkin lymphoma. Treatment with chemotherapy and/or radiation therapy for Hodgkin lymphoma may increase the risk of second cancers and other health problems for many months or years after treatment. These late effects depend on the type of treatment and the patient's age when treated, and may include: - Acute myelogenous leukemia. - Cancer of the breast, bone, cervix, gastrointestinal tract, head and neck, lung, soft tissue, and thyroid. - Heart, lung, and thyroid disease. - Avascular necrosis of bone (death of bone cells caused by lack of blood flow). - Herpes zoster (shingles) or severe infection. - Depression and fatigue. - Infertility. - Hypogonadism (low levels of testosterone and estrogen). Regular follow-up by doctors who are expert in finding and treating late effects is important for the long-term health of patients treated for Hodgkin lymphoma. Three types of standard treatment are used: Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Combination chemotherapy is treatment with more than one anticancer drug. When a pregnant woman is treated with chemotherapy for Hodgkin lymphoma, it isn't possible to protect the fetus from being exposed to the chemotherapy. Some chemotherapy regimens may cause birth defects if given in the first trimester. Vinblastine is an anticancer drug that has not been linked with birth defects when given in the second half of pregnancy. See Drugs Approved for Hodgkin Lymphoma for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat adult Hodgkin lymphoma. For a pregnant woman with Hodgkin lymphoma, radiation therapy should be postponed until after delivery, if possible, to avoid any risk to the fetus. If immediate treatment is needed, the woman may decide to continue the pregnancy and receive radiation therapy. However, lead used to shield the fetus may not protect it from scattered radiation that could possibly cause cancer in the future. Surgery Laparotomy is a procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease. If cancer is found, the tissue or organ is removed during the laparotomy. For pregnant patients with Hodgkin lymphoma, treatment options also include: Watchful waiting Watchful waiting is closely monitoring a patients condition without giving any treatment unless signs or symptoms appear or change. Delivery may be induced when the fetus is 32 to 36 weeks old, so that the mother can begin treatment. Steroid therapy Steroids are hormones made naturally in the body by the adrenal glands and by reproductive organs. Some types of steroids are made in a laboratory. Certain steroid drugs have been found to help chemotherapy work better and help stop the growth of cancer cells. Steroids can also help the lungs of the fetus develop faster than normal. This is important when delivery is induced early. See Drugs Approved for Hodgkin Lymphoma for more information. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Chemotherapy and radiation therapy with stem cell transplant High-dose chemotherapy and radiation therapy with stem cell transplant is a way of giving high doses of chemotherapy and radiation therapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After therapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. The use of lower-dose chemotherapy and radiation therapy with stem cell transplant is also being studied. Monoclonal antibody therapy Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Adult Hodgkin Lymphoma Early Favorable Hodgkin Lymphoma Treatment of early favorable Hodgkin lymphoma may include the following: - Combination chemotherapy. - Combination chemotherapy with radiation therapy to parts of the body with cancer. - Radiation therapy alone to areas of the body with cancer or to the mantle field (neck, chest, armpits). Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I adult Hodgkin lymphoma and stage II adult Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Early Unfavorable Hodgkin Lymphoma Treatment of early unfavorable Hodgkin lymphoma may include the following: - Combination chemotherapy. - Combination chemotherapy with radiation therapy to parts of the body with cancer. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I adult Hodgkin lymphoma and stage II adult Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Advanced Hodgkin Lymphoma Treatment of advanced Hodgkin lymphoma may include the following: - Combination chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III adult Hodgkin lymphoma and stage IV adult Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Adult Hodgkin Lymphoma Treatment of recurrent Hodgkin lymphoma may include the following: - Combination chemotherapy. - Combination chemotherapy followed by high-dose chemotherapy and stem cell transplant with or without radiation therapy. - Combination chemotherapy with radiation therapy to parts of the body with cancer in patients older than 60 years. - Radiation therapy with or without chemotherapy. - Chemotherapy as palliative therapy to relieve symptoms and improve quality of life. - A clinical trial of high-dose chemotherapy and stem cell transplant. - A clinical trial of lower-dose chemotherapy and radiation therapy followed by stem cell transplant. - A clinical trial of a monoclonal antibody. - A clinical trial of chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent adult Hodgkin lymphoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Treatment Options for Hodgkin Lymphoma During Pregnancy Hodgkin Lymphoma During the First Trimester of Pregnancy When Hodgkin lymphoma is diagnosed in the first trimester of pregnancy, it does not necessarily mean that the woman will be advised to end the pregnancy. Each woman's treatment will depend on the stage of the lymphoma, how fast it is growing, and her wishes. For women who choose to continue the pregnancy, treatment of Hodgkin lymphoma during the first trimester of pregnancy may include the following: - Watchful waiting when the cancer is above the diaphragm and is slow-growing. Delivery may be induced when the fetus is 32 to 36 weeks old so the mother can begin treatment. - Radiation therapy above the diaphragm. (A lead shield is used to protect the fetus from the radiation as much as possible.) - Systemic chemotherapy using one or more drugs. Hodgkin Lymphoma During the Second Half of Pregnancy When Hodgkin lymphoma is diagnosed in the second half of pregnancy, most women can delay treatment until after the baby is born. Treatment of Hodgkin lymphoma during the second half of pregnancy may include the following: - Watchful waiting, with plans to induce delivery when the fetus is 32 to 36 weeks old. - Systemic chemotherapy using one or more drugs. - Steroid therapy. - Radiation therapy to relieve breathing problems caused by a large tumor in the chest.
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Ovarian Germ Cell Tumors
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information
What is (are) Ovarian Germ Cell Tumors ?
Key Points - Ovarian germ cell tumor is a disease in which malignant (cancer) cells form in the germ (egg) cells of the ovary. - Signs of ovarian germ cell tumor are swelling of the abdomen or vaginal bleeding after menopause. - Tests that examine the ovaries, pelvic area, blood, and ovarian tissue are used to detect (find) and diagnose ovarian germ cell tumor. - Certain factors affect prognosis (chance of recovery and treatment options). Ovarian germ cell tumor is a disease in which malignant (cancer) cells form in the germ (egg) cells of the ovary. Germ cell tumors begin in the reproductive cells (egg or sperm) of the body. Ovarian germ cell tumors usually occur in teenage girls or young women and most often affect just one ovary. The ovaries are a pair of organs in the female reproductive system. They are in the pelvis, one on each side of the uterus (the hollow, pear-shaped organ where a fetus grows). Each ovary is about the size and shape of an almond. The ovaries make eggs and female hormones. Ovarian germ cell tumor is a general name that is used to describe several different types of cancer. The most common ovarian germ cell tumor is called dysgerminoma. See the following PDQ summaries for information about other types of ovarian tumors: - Ovarian Epithelial, Fallopian Tube, and Primary Peritoneal Cancer Treatment - Ovarian Low Malignant Potential Tumors Treatment
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Ovarian Germ Cell Tumors
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symptoms
What are the symptoms of Ovarian Germ Cell Tumors ?
Signs of ovarian germ cell tumor are swelling of the abdomen or vaginal bleeding after menopause. Ovarian germ cell tumors can be hard to diagnose (find) early. Often there are no symptoms in the early stages, but tumors may be found during regular gynecologic exams (checkups). Check with your doctor if you have either of the following: - Swollen abdomen without weight gain in other parts of the body. - Bleeding from the vagina after menopause (when you are no longer having menstrual periods).
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Ovarian Germ Cell Tumors
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exams and tests
How to diagnose Ovarian Germ Cell Tumors ?
Tests that examine the ovaries, pelvic area, blood, and ovarian tissue are used to detect (find) and diagnose ovarian germ cell tumor. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Pelvic exam : An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test of the cervix is usually done. The doctor or nurse also inserts one or two lubricated, gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas. - Laparotomy : A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - Serum tumor marker test : A procedure in which a sample of blood is checked to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers. An increased level of alpha fetoprotein (AFP) or human chorionic gonadotropin (HCG) in the blood may be a sign of ovarian germ cell tumor.
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Ovarian Germ Cell Tumors
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outlook
What is the outlook for Ovarian Germ Cell Tumors ?
Certain factors affect prognosis (chance of recovery and treatment options). The prognosis (chance of recovery) and treatment options depend on the following: - The type of cancer. - The size of the tumor. - The stage of cancer (whether it affects part of the ovary, involves the whole ovary, or has spread to other places in the body). - The way the cancer cells look under a microscope. - The patients general health. Ovarian germ cell tumors are usually cured if found and treated early.
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Ovarian Germ Cell Tumors
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stages
What are the stages of Ovarian Germ Cell Tumors ?
Key Points - After ovarian germ cell tumor has been diagnosed, tests are done to find out if cancer cells have spread within the ovary or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for ovarian germ cell tumors: - Stage I - Stage II - Stage III - Stage IV After ovarian germ cell tumor has been diagnosed, tests are done to find out if cancer cells have spread within the ovary or to other parts of the body. The process used to find out whether cancer has spread within the ovary or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. Unless a doctor is sure the cancer has spread from the ovaries to other parts of the body, an operation called a laparotomy is done to see if the cancer has spread. The doctor must cut into the abdomen and carefully look at all the organs to see if they have cancer in them. The doctor will cut out small pieces of tissue so they can be checked under a microscope for signs of cancer. The doctor may also wash the abdominal cavity with fluid, which is also checked under a microscope to see if it has cancer cells in it. Usually the doctor will remove the cancer and other organs that have cancer in them during the laparotomy. It is important to know the stage in order to plan treatment. Many of the tests used to diagnose ovarian germ cell tumor are also used for staging. The following tests and procedures may also be used for staging: - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Transvaginal ultrasound exam: A procedure used to examine the vagina, uterus, fallopian tubes, and bladder. An ultrasound transducer (probe) is inserted into the vagina and used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The doctor can identify tumors by looking at the sonogram. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of tumor as the primary tumor. For example, if an ovarian germ cell tumor spreads to the liver, the tumor cells in the liver are actually cancerous ovarian germ cells. The disease is metastatic ovarian germ cell tumor, not liver cancer. The following stages are used for ovarian germ cell tumors: Stage I In stage I, cancer is found in one or both ovaries. Stage I is divided into stage IA, stage IB, and stage IC. - Stage IA: Cancer is found inside a single ovary. - Stage IB: Cancer is found inside both ovaries. - Stage IC: Cancer is found inside one or both ovaries and one of the following is true: - cancer is also found on the outside surface of one or both ovaries; or - the capsule (outer covering) of the ovary has ruptured (broken open); or - cancer cells are found in the fluid of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen) or in washings of the peritoneum (tissue lining the peritoneal cavity). Stage II In stage II, cancer is found in one or both ovaries and has spread into other areas of the pelvis. Stage II is divided into stage IIA, stage IIB, and stage IIC. - Stage IIA: Cancer has spread to the uterus and/or fallopian tubes (the long slender tubes through which eggs pass from the ovaries to the uterus). - Stage IIB: Cancer has spread to other tissue within the pelvis. - Stage IIC: Cancer is found inside one or both ovaries and has spread to the uterus and/or fallopian tubes, or to other tissue within the pelvis. Also, one of the following is true: - cancer is found on the outside surface of one or both ovaries; or - the capsule (outer covering) of the ovary has ruptured (broken open); or - cancer cells are found in the fluid of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen) or in washings of the peritoneum (tissue lining the peritoneal cavity). Stage III In stage III, cancer is found in one or both ovaries and has spread outside the pelvis to other parts of the abdomen and/or nearby lymph nodes. Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. - Stage IIIA: The tumor is found in the pelvis only, but cancer cells that can be seen only with a microscope have spread to the surface of the peritoneum (tissue that lines the abdominal wall and covers most of the organs in the abdomen), the small intestines, or the tissue that connects the small intestines to the wall of the abdomen. - Stage IIIB: Cancer has spread to the peritoneum and the cancer in the peritoneum is 2 centimeters or smaller. - Stage IIIC: Cancer has spread to the peritoneum and the cancer in the peritoneum is larger than 2 centimeters and/or cancer has spread to lymph nodes in the abdomen. Cancer that has spread to the surface of the liver is also considered stage III ovarian cancer. Stage IV In stage IV, cancer has spread beyond the abdomen to other parts of the body, such as the lungs or tissue inside the liver. Cancer cells in the fluid around the lungs is also considered stage IV ovarian cancer.
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Ovarian Germ Cell Tumors
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treatment
What are the treatments for Ovarian Germ Cell Tumors ?
Key Points - There are different types of treatment for patients with ovarian germ cell tumors. - Four types of standard treatment are used: - Surgery - Observation - Chemotherapy - Radiation therapy - New types of treatment are being tested in clinical trials. - High-dose chemotherapy with bone marrow transplant - New treatment options - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with ovarian germ cell tumors. Different types of treatment are available for patients with ovarian germ cell tumor. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Four types of standard treatment are used: Surgery Surgery is the most common treatment of ovarian germ cell tumor. A doctor may take out the cancer using one of the following types of surgery. - Unilateral salpingo-oophorectomy: A surgical procedure to remove one ovary and one fallopian tube. - Total hysterectomy: A surgical procedure to remove the uterus, including the cervix. If the uterus and cervix are taken out through the vagina, the operation is called a vaginal hysterectomy. If the uterus and cervix are taken out through a large incision (cut) in the abdomen, the operation is called a total abdominal hysterectomy. If the uterus and cervix are taken out through a small incision (cut) in the abdomen using a laparoscope, the operation is called a total laparoscopic hysterectomy. - Bilateral salpingo-oophorectomy: A surgical procedure to remove both ovaries and both fallopian tubes. - Tumor debulking: A surgical procedure in which as much of the tumor as possible is removed. Some tumors cannot be completely removed. Even if the doctor removes all the cancer that can be seen at the time of the operation, some patients may be offered chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. After chemotherapy for an ovarian germ cell tumor, a second-look laparotomy may be done. This is similar to the laparotomy that is done to find out the stage of the cancer. Second-look laparotomy is a surgical procedure to find out if tumor cells are left after primary treatment. During this procedure, the doctor will take samples of lymph nodes and other tissues in the abdomen to see if any cancer is left. This procedure is not done for dysgerminomas. Observation Observation is closely watching a patients condition without giving any treatment unless signs or symptoms appear or change. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Ovarian, Fallopian Tube, or Primary Peritoneal Cancer for more information. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat ovarian germ cell tumors. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with bone marrow transplant High-dose chemotherapy with bone marrow transplant is a method of giving very high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. New treatment options Combination chemotherapy (the use of more than one anticancer drug) is being tested in clinical trials. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options By Stage Stage I Ovarian Germ Cell Tumors Treatment depends on whether the tumor is a dysgerminoma or another type of ovarian germ cell tumor. Treatment of dysgerminoma may include the following: - Unilateral salpingo-oophorectomy with or without lymphangiography or CT scan. - Unilateral salpingo-oophorectomy followed by observation. - Unilateral salpingo-oophorectomy followed by radiation therapy. - Unilateral salpingo-oophorectomy followed by chemotherapy. Treatment of other ovarian germ cell tumors may be either: - unilateral salpingo-oophorectomy followed by careful observation; or - unilateral salpingo-oophorectomy, sometimes followed by combination chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I ovarian germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Ovarian Germ Cell Tumors Treatment depends on whether the tumor is a dysgerminoma or another type of ovarian germ cell tumor. Treatment of dysgerminoma may be either: - total abdominal hysterectomy and bilateral salpingo-oophorectomy followed by radiation therapy or combination chemotherapy; or - unilateral salpingo-oophorectomy followed by chemotherapy. Treatment of other ovarian germ cell tumors may include the following: - Unilateral salpingo-oophorectomy followed by combination chemotherapy. - Second-look laparotomy (surgery done after primary treatment to see if tumor cells remain). - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II ovarian germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Ovarian Germ Cell Tumors Treatment depends on whether the tumor is a dysgerminoma or another type of ovarian germ cell tumor. Treatment of dysgerminoma may include the following: - Total abdominal hysterectomy and bilateral salpingo-oophorectomy, with removal of as much of the cancer in the pelvis and abdomen as possible. - Unilateral salpingo-oophorectomy followed by chemotherapy. Treatment of other ovarian germ cell tumors may include the following: - Total abdominal hysterectomy and bilateral salpingo-oophorectomy, with removal of as much of the cancer in the pelvis and abdomen as possible. Chemotherapy will be given before and/or after surgery. - Unilateral salpingo-oophorectomy followed by chemotherapy. - Second-look laparotomy (surgery done after primary treatment to see if tumor cells remain). - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III ovarian germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Ovarian Germ Cell Tumors Treatment depends on whether the tumor is a dysgerminoma or another type of ovarian germ cell tumor. Treatment of dysgerminoma may include the following: - Total abdominal hysterectomy and bilateral salpingo-oophorectomy followed by chemotherapy, with removal of as much of the cancer in the pelvis and abdomen as possible. - Unilateral salpingo-oophorectomy followed by chemotherapy. Treatment of other ovarian germ cell tumors may include the following: - Total abdominal hysterectomy and bilateral salpingo-oophorectomy, with removal of as much of the cancer in the pelvis and abdomen as possible. Chemotherapy will be given before and/or after surgery. - Unilateral salpingo-oophorectomy followed by chemotherapy. - Second-look laparotomy (surgery done after primary treatment to see if tumor cells remain). - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV ovarian germ cell tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
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Ovarian Germ Cell Tumors
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research
what research (or clinical trials) is being done for Ovarian Germ Cell Tumors ?
New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. High-dose chemotherapy with bone marrow transplant High-dose chemotherapy with bone marrow transplant is a method of giving very high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the bodys blood cells. New treatment options Combination chemotherapy (the use of more than one anticancer drug) is being tested in clinical trials. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
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Prostate Cancer
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information
What is (are) Prostate Cancer ?
Key Points - Prostate cancer is a disease in which malignant (cancer) cells form in the tissues of the prostate. - Prostate cancer is the most common nonskin cancer among men in the United States. - Different factors increase or decrease the risk of developing prostate cancer. Prostate cancer is a disease in which malignant (cancer) cells form in the tissues of the prostate. The prostate is a gland in the male reproductive system located just below the bladder (the organ that collects and empties urine) and in front of the rectum (the lower part of the intestine). It is about the size of a walnut and surrounds part of the urethra (the tube that empties urine from the bladder). The prostate gland produces fluid that makes up part of semen. As men age, the prostate may get bigger. A bigger prostate may block the flow of urine from the bladder and cause problems with sexual function. This condition is called benign prostatic hyperplasia (BPH), and although it is not cancer, surgery may be needed to correct it. The symptoms of benign prostatic hyperplasia or of other problems in the prostate may be similar to symptoms of prostate cancer. See the following PDQ summaries for more information about prostate cancer: - Prostate Cancer Prevention - Prostate Cancer Treatment
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Prostate Cancer
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susceptibility
Who is at risk for Prostate Cancer? ?
Prostate cancer is the most common nonskin cancer among men in the United States. Prostate cancer is found mainly in older men. Although the number of men with prostate cancer is large, most men diagnosed with this disease do not die from it. Prostate cancer causes more deaths in men than any other cancer except lung cancer and colorectal cancer. Prostate cancer occurs more often in African-American men than in white men. African-American men with prostate cancer are more likely to die from the disease than white men with prostate cancer.
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Prostate Cancer
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susceptibility
Who is at risk for Prostate Cancer? ?
Different factors increase or decrease the risk of developing prostate cancer. Anything that increases a person's chance of developing a disease is called a risk factor. Anything that decreases your chance of getting a disease is called a protective factor. For information about risk factors and protective factors for prostate cancer, see the PDQ summary on Prostate Cancer Prevention.
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Ovarian Low Malignant Potential Tumors
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information
What is (are) Ovarian Low Malignant Potential Tumors ?
Key Points - Ovarian low malignant potential tumor is a disease in which abnormal cells form in the tissue covering the ovary. - Signs and symptoms of ovarian low malignant potential tumor include pain or swelling in the abdomen. - Tests that examine the ovaries are used to detect (find), diagnose, and stage ovarian low malignant potential tumor. - Certain factors affect prognosis (chance of recovery) and treatment options. Ovarian low malignant potential tumor is a disease in which abnormal cells form in the tissue covering the ovary. Ovarian low malignant potential tumors have abnormal cells that may become cancer, but usually do not. This disease usually remains in the ovary. When disease is found in one ovary, the other ovary should also be checked carefully for signs of disease. The ovaries are a pair of organs in the female reproductive system. They are in the pelvis, one on each side of the uterus (the hollow, pear-shaped organ where a fetus grows). Each ovary is about the size and shape of an almond. The ovaries make eggs and female hormones.
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Ovarian Low Malignant Potential Tumors
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symptoms
What are the symptoms of Ovarian Low Malignant Potential Tumors ?
Signs and symptoms of ovarian low malignant potential tumor include pain or swelling in the abdomen.Ovarian low malignant potential tumor may not cause early signs or symptoms. If you do have signs or symptoms, they may include the following: - Pain or swelling in the abdomen. - Pain in the pelvis. - Gastrointestinal problems, such as gas, bloating, or constipation. These signs and symptoms may be caused by other conditions. If they get worse or do not go away on their own, check with your doctor.
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Ovarian Low Malignant Potential Tumors
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exams and tests
How to diagnose Ovarian Low Malignant Potential Tumors ?
Tests that examine the ovaries are used to detect (find), diagnose, and stage ovarian low malignant potential tumor. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Pelvic exam : An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test of the cervix is usually done. The doctor or nurse also inserts one or two lubricated, gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.Other patients may have a transvaginal ultrasound. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - CA 125 assay : A test that measures the level of CA 125 in the blood. CA 125 is a substance released by cells into the bloodstream. An increased CA 125 level is sometimes a sign of cancer or other condition. - Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The tissue is usually removed during surgery to remove the tumor.
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Ovarian Low Malignant Potential Tumors
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outlook
What is the outlook for Ovarian Low Malignant Potential Tumors ?
Certain factors affect prognosis (chance of recovery) and treatment options.The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the disease (whether it affects part of the ovary, involves the whole ovary, or has spread to other places in the body). - What type of cells make up the tumor. - The size of the tumor. - The patients general health. Patients with ovarian low malignant potential tumors have a good prognosis, especially when the tumor is found early.
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Ovarian Low Malignant Potential Tumors
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stages
What are the stages of Ovarian Low Malignant Potential Tumors ?
Key Points - After ovarian low malignant potential tumor has been diagnosed, tests are done to find out if abnormal cells have spread within the ovary or to other parts of the body. - The following stages are used for ovarian low malignant potential tumor: - Stage I - Stage II - Stage III - Stage IV After ovarian low malignant potential tumor has been diagnosed, tests are done to find out if abnormal cells have spread within the ovary or to other parts of the body. The process used to find out whether abnormal cells have spread within the ovary or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. Certain tests or procedures are used for staging. Staging laparotomy (a surgical incision made in the wall of the abdomen to remove ovarian tissue) may be used. Most patients are diagnosed with stage I disease. The following stages are used for ovarian low malignant potential tumor: Stage I In stage I, the tumor is found in one or both ovaries. Stage I is divided into stage IA, stage IB, and stage IC. - Stage IA: The tumor is found inside a single ovary. - Stage IB: The tumor is found inside both ovaries. - Stage IC: The tumor is found inside one or both ovaries and one of the following is true: - tumor cells are found on the outside surface of one or both ovaries; or - the capsule (outer covering) of the ovary has ruptured (broken open); or - tumor cells are found in the fluid of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen) or in washings of the peritoneum (tissue lining the peritoneal cavity). Stage II In stage II, the tumor is found in one or both ovaries and has spread into other areas of the pelvis. Stage II is divided into stage IIA, stage IIB, and stage IIC. - Stage IIA: The tumor has spread to the uterus and/or fallopian tubes (the long slender tubes through which eggs pass from the ovaries to the uterus). - Stage IIB: The tumor has spread to other tissue within the pelvis. - Stage IIC: The tumor is found inside one or both ovaries and has spread to the uterus and/or fallopian tubes, or to other tissue within the pelvis. Also, one of the following is true: - tumor cells are found on the outside surface of one or both ovaries; or - the capsule (outer covering) of the ovary has ruptured (broken open); or - tumor cells are found in the fluid of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen) or in washings of the peritoneum (tissue lining the peritoneal cavity). Stage III In stage III, the tumor is found in one or both ovaries and has spread outside the pelvis to other parts of the abdomen and/or nearby lymph nodes. Stage III is divided into stage IIIA, stage IIIB, and stage IIIC. - Stage IIIA: The tumor is found in the pelvis only, but tumor cells that can be seen only with a microscope have spread to the surface of the peritoneum (tissue that lines the abdominal wall and covers most of the organs in the abdomen), the small intestines, or the tissue that connects the small intestines to the wall of the abdomen. - Stage IIIB: The tumor has spread to the peritoneum and the tumor in the peritoneum is 2 centimeters or smaller. - Stage IIIC: The tumor has spread to the peritoneum and the tumor in the peritoneum is larger than 2 centimeters and/or has spread to lymph nodes in the abdomen. The spread of tumor cells to the surface of the liver is also considered stage III disease. Stage IV In stage IV, tumor cells have spread beyond the abdomen to other parts of the body, such as the lungs or tissue inside the liver. Tumor cells in the fluid around the lungs is also considered stage IV disease. Ovarian low malignant potential tumors almost never reach stage IV.
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Ovarian Low Malignant Potential Tumors
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treatment
What are the treatments for Ovarian Low Malignant Potential Tumors ?
Key Points - There are different types of treatment for patients with ovarian low malignant potential tumor. - Two types of standard treatment are used: - Surgery - Chemotherapy - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their treatment. - Follow-up tests may be needed. There are different types of treatment for patients with ovarian low malignant potential tumor. Different types of treatment are available for patients with ovarian low malignant potential tumor. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer, tumors, and related conditions. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Two types of standard treatment are used: Surgery The type of surgery (removing the tumor in an operation) depends on the size and spread of the tumor and the womans plans for having children. Surgery may include the following: - Unilateral salpingo-oophorectomy: Surgery to remove one ovary and one fallopian tube. - Bilateral salpingo-oophorectomy: Surgery to remove both ovaries and both fallopian tubes. - Total hysterectomy and bilateral salpingo-oophorectomy: Surgery to remove the uterus, cervix, and both ovaries and fallopian tubes. If the uterus and cervix are taken out through the vagina, the operation is called a vaginal hysterectomy. If the uterus and cervix are taken out through a large incision (cut) in the abdomen, the operation is called a total abdominal hysterectomy. If the uterus and cervix are taken out through a small incision (cut) in the abdomen using a laparoscope, the operation is called a total laparoscopic hysterectomy. - Partial oophorectomy: Surgery to remove part of one ovary or part of both ovaries. - Omentectomy: Surgery to remove the omentum (a piece of the tissue lining the abdominal wall). Even if the doctor removes all disease that can be seen at the time of the operation, the patient may be given chemotherapy after surgery to kill any tumor cells that are left. Treatment given after the surgery, to lower the risk that the tumor will come back, is called adjuvant therapy. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI Web site. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the medical research process. Clinical trials are done to find out if new treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for disease are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way diseases will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose disease has not gotten better. There are also clinical trials that test new ways to stop a disease from recurring (coming back) or reduce the side effects of treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database. Follow-up tests may be needed. Some of the tests that were done to diagnose the disease may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the disease has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Ovarian Low Malignant Potential Tumors Early Stage Ovarian Low Malignant Potential Tumors (Stage I and II) Surgery is the standard treatment for early stage ovarian low malignant potential tumor. The type of surgery usually depends on whether a woman plans to have children. For women who plan to have children, surgery is either: - unilateral salpingo-oophorectomy; or - partial oophorectomy. To prevent recurrence of disease, most doctors recommend surgery to remove the remaining ovarian tissue when a woman no longer plans to have children. For women who do not plan to have children, treatment may be hysterectomy and bilateral salpingo-oophorectomy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I borderline ovarian surface epithelial-stromal tumor and stage II borderline ovarian surface epithelial-stromal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Late Stage Ovarian Low Malignant Potential Tumors (Stage III and IV) Treatment for late stage ovarian low malignant potential tumor may be hysterectomy, bilateral salpingo-oophorectomy, and omentectomy. A lymph node dissection may also be done. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III borderline ovarian surface epithelial-stromal tumor and stage IV borderline ovarian surface epithelial-stromal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Recurrent Ovarian Low Malignant Potential Tumors Treatment for recurrent ovarian low malignant potential tumor may include the following: - Surgery. - Surgery followed by chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent borderline ovarian surface epithelial-stromal tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
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Ovarian Low Malignant Potential Tumors
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research
what research (or clinical trials) is being done for Ovarian Low Malignant Potential Tumors ?
New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI Web site. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the medical research process. Clinical trials are done to find out if new treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for disease are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way diseases will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose disease has not gotten better. There are also clinical trials that test new ways to stop a disease from recurring (coming back) or reduce the side effects of treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.
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Merkel Cell Carcinoma
1
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information
What is (are) Merkel Cell Carcinoma ?
Key Points - Merkel cell carcinoma is a very rare disease in which malignant (cancer) cells form in the skin. - Sun exposure and having a weak immune system can affect the risk of Merkel cell carcinoma. - Merkel cell carcinoma usually appears as a single painless lump on sun-exposed skin. - Tests and procedures that examine the skin are used to detect (find) and diagnose Merkel cell carcinoma. - Certain factors affect prognosis (chance of recovery) and treatment options. Merkel cell carcinoma is a very rare disease in which malignant (cancer) cells form in the skin. Merkel cells are found in the top layer of the skin. These cells are very close to the nerve endings that receive the sensation of touch. Merkel cell carcinoma, also called neuroendocrine carcinoma of the skin or trabecular cancer, is a very rare type of skin cancer that forms when Merkel cells grow out of control. Merkel cell carcinoma starts most often in areas of skin exposed to the sun, especially the head and neck, as well as the arms, legs, and trunk. Merkel cell carcinoma tends to grow quickly and to metastasize (spread) at an early stage. It usually spreads first to nearby lymph nodes and then may spread to lymph nodes or skin in distant parts of the body, lungs, brain, bones, or other organs.
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Merkel Cell Carcinoma
2
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susceptibility
Who is at risk for Merkel Cell Carcinoma? ?
un exposure and having a weak immune system can affect the risk of Merkel cell carcinoma. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for Merkel cell carcinoma include the following: - Being exposed to a lot of natural sunlight. - Being exposed to artificial sunlight, such as from tanning beds or psoralen and ultraviolet A (PUVA) therapy for psoriasis. - Having an immune system weakened by disease, such as chronic lymphocytic leukemia or HIV infection. - Taking drugs that make the immune system less active, such as after an organ transplant. - Having a history of other types of cancer. - Being older than 50 years, male, or white.
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Merkel Cell Carcinoma
3
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symptoms
What are the symptoms of Merkel Cell Carcinoma ?
Merkel cell carcinoma usually appears as a single painless lump on sun-exposed skin. This and other changes in the skin may be caused by Merkel cell carcinoma or by other conditions. Check with your doctor if you see changes in your skin. Merkel cell carcinoma usually appears on sun-exposed skin as a single lump that is: - Fast-growing. - Painless. - Firm and dome-shaped or raised. - Red or violet in color.
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Merkel Cell Carcinoma
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exams and tests
How to diagnose Merkel Cell Carcinoma ?
Tests and procedures that examine the skin are used to detect (find) and diagnose Merkel cell carcinoma. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Full-body skin exam: A doctor or nurse checks the skin for bumps or spots that look abnormal in color, size, shape, or texture. The size, shape, and texture of the lymph nodes will also be checked. - Skin biopsy : The removal of skin cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.
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Merkel Cell Carcinoma
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outlook
What is the outlook for Merkel Cell Carcinoma ?
Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer (the size of the tumor and whether it has spread to the lymph nodes or other parts of the body). - Where the cancer is in the body. - Whether the cancer has just been diagnosed or has recurred (come back). - The patient's age and general health. Prognosis also depends on how deeply the tumor has grown into the skin.
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Merkel Cell Carcinoma
6
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stages
What are the stages of Merkel Cell Carcinoma ?
Key Points - After Merkel cell carcinoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for Merkel cell carcinoma: - Stage 0 (carcinoma in situ) - Stage IA - Stage IB - Stage IIA - Stage IIB - Stage IIC - Stage IIIA - Stage IIIB - Stage IV After Merkel cell carcinoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body. The process used to find out if cancer has spread to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. A CT scan of the chest and abdomen may be used to check for primary small cell lung cancer, or to find Merkel cell carcinoma that has spread. A CT scan of the head and neck may also be used to find Merkel cell carcinoma that has spread to the lymph nodes. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Lymph node biopsy : There are two main types of lymph node biopsy used to stage Merkel cell carcinoma. - Sentinel lymph node biopsy : The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. - Lymph node dissection : A surgical procedure in which the lymph nodes are removed and a sample of tissue is checked under a microscope for signs of cancer. For a regional lymph node dissection, some of the lymph nodes in the tumor area are removed. For a radical lymph node dissection, most or all of the lymph nodes in the tumor area are removed. This procedure is also called lymphadenectomy. - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if Merkel cell carcinoma spreads to the liver, the cancer cells in the liver are actually cancerous Merkel cells. The disease is metastatic Merkel cell carcinoma, not liver cancer. The following stages are used for Merkel cell carcinoma: Stage 0 (carcinoma in situ) In stage 0, the tumor is a group of abnormal cells that remain in the place where they first formed and have not spread. These abnormal cells may become cancer and spread to lymph nodes or distant parts of the body. Stage IA In stage IA, the tumor is 2 centimeters or smaller at its widest point and no cancer is found when the lymph nodes are checked under a microscope. Stage IB In stage IB, the tumor is 2 centimeters or smaller at its widest point and no swollen lymph nodes are found by a physical exam or imaging tests. Stage IIA In stage IIA, the tumor is larger than 2 centimeters and no cancer is found when the lymph nodes are checked under a microscope. Stage IIB In stage IIB, the tumor is larger than 2 centimeters and no swollen lymph nodes are found by a physical exam or imaging tests. Stage IIC In stage IIC, the tumor may be any size and has spread to nearby bone, muscle, connective tissue, or cartilage. It has not spread to lymph nodes or distant parts of the body. Stage IIIA In stage IIIA, the tumor may be any size and may have spread to nearby bone, muscle, connective tissue, or cartilage. Cancer is found in the lymph nodes when they are checked under a microscope. Stage IIIB In stage IIIB, the tumor may be any size and may have spread to nearby bone, muscle, connective tissue, or cartilage. Cancer has spread to the lymph nodes near the tumor and is found by a physical exam or imaging test. The lymph nodes are removed and cancer is found in the lymph nodes when they are checked under a microscope. There may also be a second tumor, which is either: - Between the primary tumor and nearby lymph nodes; or - Farther away from the center of the body than the primary tumor is. Stage IV In stage IV, the tumor may be any size and has spread to distant parts of the body, such as the liver, lung, bone, or brain.
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Merkel Cell Carcinoma
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treatment
What are the treatments for Merkel Cell Carcinoma ?
Key Points - There are different types of treatment for patients with Merkel cell carcinoma. - Three types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - New types of treatment are being tested in clinical trials. - Treatment for Merkel cell carcinoma may cause side effects. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with Merkel cell carcinoma. Different types of treatments are available for patients with Merkel cell carcinoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Three types of standard treatment are used: Surgery One or more of the following surgical procedures may be used to treat Merkel cell carcinoma: - Wide local excision: The cancer is cut from the skin along with some of the tissue around it. A sentinel lymph node biopsy may be done during the wide local excision procedure. If there is cancer in the lymph nodes, a lymph node dissection also may be done. - Lymph node dissection: A surgical procedure in which the lymph nodes are removed and a sample of tissue is checked under a microscope for signs of cancer. For a regional lymph node dissection, some of the lymph nodes in the tumor area are removed; for a radical lymph node dissection, most or all of the lymph nodes in the tumor area are removed. This procedure is also called lymphadenectomy. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat Merkel cell carcinoma, and may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. New types of treatment are being tested in clinical trials. Treatment for Merkel cell carcinoma may cause side effects. For information about side effects caused by treatment for cancer, see our Side Effects page. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage I and Stage II Merkel Cell Carcinoma Treatment of stage I and stage II Merkel cell carcinoma may include the following: - Surgery to remove the tumor, such as wide local excision with or without lymph node dissection. - Radiation therapy after surgery. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I neuroendocrine carcinoma of the skin and stage II neuroendocrine carcinoma of the skin. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Merkel Cell Carcinoma Treatment of stage III Merkel cell carcinoma may include the following: - Wide local excision with or without lymph node dissection. - Radiation therapy. - A clinical trial of chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III neuroendocrine carcinoma of the skin. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Merkel Cell Carcinoma Treatment of stage IV Merkel cell carcinoma may include the following as palliative treatment to relieve symptoms and improve quality of life: - Chemotherapy. - Surgery. - Radiation therapy. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV neuroendocrine carcinoma of the skin. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
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Merkel Cell Carcinoma
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research
what research (or clinical trials) is being done for Merkel Cell Carcinoma ?
New types of treatment are being tested in clinical trials. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
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Breast Cancer
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information
What is (are) Breast Cancer ?
Key Points - Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. - Breast cancer is the second leading cause of death from cancer in American women. - Different factors increase or decrease the risk of breast cancer. Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. The breast is made up of lobes and ducts. Each breast has 15 to 20 sections called lobes, which have many smaller sections called lobules. Lobules end in dozens of tiny bulbs that can produce milk. The lobes, lobules, and bulbs are linked by thin tubes called ducts. Each breast also contains blood vessels and lymph vessels. The lymph vessels carry an almost colorless fluid called lymph. Lymph vessels lead to organs called lymph nodes. Lymph nodes are small bean-shaped structures that are found throughout the body. They filter substances in lymph and help fight infection and disease. Clusters of lymph nodes are found near the breast in the axilla (under the arm), above the collarbone, and in the chest. See the following PDQ summaries for more information about breast cancer: - Breast Cancer Prevention - Breast Cancer Treatment - Genetics of Breast and Gynecologic Cancers Breast cancer is the second leading cause of death from cancer in American women. Women in the United States get breast cancer more than any other type of cancer except for skin cancer. Breast cancer is second only to lung cancer as a cause of cancer death in women. Breast cancer occurs more often in white women than in black women. However, black women are more likely than white women to die from the disease. Breast cancer occurs in men also, but the number of cases is small.
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Breast Cancer
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susceptibility
Who is at risk for Breast Cancer? ?
Different factors increase or decrease the risk of breast cancer. Anything that increases your chance of getting a disease is called a risk factor. Anything that decreases your chance of getting a disease is called a protective factor. For information about risk factors and protective factors for breast cancer, see the PDQ summary on Breast Cancer Prevention.
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Breast Cancer
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research
what research (or clinical trials) is being done for Breast Cancer ?
Other screening tests are being studied in clinical trials. Thermography Thermography is a procedure in which a special camera that senses heat is used to record the temperature of the skin that covers the breasts. A computer makes a map of the breast showing the changes in temperature. Tumors can cause temperature changes that may show up on the thermogram. There have been no clinical trials of thermography to find out how well it detects breast cancer or if having the procedure decreases the risk of dying from breast cancer. Tissue sampling Breast tissue sampling is taking cells from breast tissue to check under a microscope. Abnormal cells in breast fluid have been linked to an increased risk of breast cancer in some studies. Scientists are studying whether breast tissue sampling can be used to find breast cancer at an early stage or predict the risk of developing breast cancer. Three ways of taking tissue samples are being studied: - Fine-needle aspiration: A thin needle is inserted into the breast tissue around the areola (darkened area around the nipple) to take out a sample of cells and fluid. - Nipple aspiration: The use of gentle suction to collect fluid through the nipple. This is done with a device similar to the breast pumps used by women who are breast-feeding. - Ductal lavage: A hair-size catheter (tube) is inserted into the nipple and a small amount of salt water is released into the duct. The water picks up breast cells and is removed. Screening clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI website.
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Wilms Tumor and Other Childhood Kidney Tumors
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information
What is (are) Wilms Tumor and Other Childhood Kidney Tumors ?
Key Points - Childhood kidney tumors are diseases in which malignant (cancer) cells form in the tissues of the kidney. - There are many types of childhood kidney tumors. - Wilms Tumor - Renal Cell Cancer (RCC) - Rhabdoid Tumor of the Kidney - Clear Cell Sarcoma of the Kidney - Congenital Mesoblastic Nephroma - Ewing Sarcoma of the Kidney - Primary Renal Myoepithelial Carcinoma - Cystic Partially Differentiated Nephroblastoma - Multilocular Cystic Nephroma - Primary Renal Synovial Sarcoma - Anaplastic Sarcoma of the Kidney - Nephroblastomatosis is not cancer but may become Wilms tumor. - Having certain genetic syndromes or other conditions can increase the risk of Wilms tumor. - Tests are used to screen for Wilms tumor. - Having certain conditions may increase the risk of renal cell cancer. - Treatment for Wilms tumor and other childhood kidney tumors may include genetic counseling. - Signs of Wilms tumor and other childhood kidney tumors include a lump in the abdomen and blood in the urine. - Tests that examine the kidney and the blood are used to detect (find) and diagnose Wilms tumor and other childhood kidney tumors. - Certain factors affect prognosis (chance of recovery) and treatment options. Childhood kidney tumors are diseases in which malignant (cancer) cells form in the tissues of the kidney. There are two kidneys, one on each side of the backbone, above the waist. Tiny tubules in the kidneys filter and clean the blood. They take out waste products and make urine. The urine passes from each kidney through a long tube called a ureter into the bladder. The bladder holds the urine until it passes through the urethra and leaves the body. There are many types of childhood kidney tumors. Wilms Tumor In Wilms tumor, one or more tumors may be found in one or both kidneys. Wilms tumor may spread to the lungs, liver, bone, brain, or nearby lymph nodes. In children and adolescents younger than 15 years old, most kidney cancers are Wilms tumors. Renal Cell Cancer (RCC) Renal cell cancer is rare in children and adolescents younger than 15 years old. It is much more common in adolescents between 15 and 19 years old. Children and adolescents are more likely to be diagnosed with a large renal cell tumor or cancer that has spread. Renal cell cancers may spread to the lungs, liver, or lymph nodes. Renal cell cancer may also be called renal cell carcinoma. Rhabdoid Tumor of the Kidney Rhabdoid tumor of the kidney is a type of kidney cancer that occurs mostly in infants and young children. It is often advanced at the time of diagnosis. Rhabdoid tumor of the kidney grows and spreads quickly, often to the lungs or brain. Children with a certain change in the SMARCB1 gene are checked regularly to see if a rhabdoid tumor has formed in the kidney or has spread to the brain: - Children younger than one year old have an ultrasound of the abdomen every two to three months and an ultrasound of the head every month. - Children one to four years old have an ultrasound of the abdomen and an MRI of the brain and spine every three months. Clear Cell Sarcoma of the Kidney Clear cell sarcoma of the kidney is a type of kidney tumor that may spread to the lung, bone, brain, or soft tissue. When it recurs (comes back) after treatment, it often recurs in the brain or lung. Congenital Mesoblastic Nephroma Congenital mesoblastic nephroma is a tumor of the kidney that is often diagnosed during the first year of life. It can usually be cured. Ewing Sarcoma of the Kidney Ewing sarcoma (previously called neuroepithelial tumor) of the kidney is rare and usually occurs in young adults. These tumors grow and spread to other parts of the body quickly. Primary Renal Myoepithelial Carcinoma Primary renal myoepithelial carcinoma is a rare type of cancer that usually affects soft tissues, but sometimes forms in the internal organs (such as the kidney). This type of cancer grows and spreads quickly. Cystic Partially Differentiated Nephroblastoma Cystic partially differentiated nephroblastoma is a very rare type of Wilms tumor made up of cysts. Multilocular Cystic Nephroma Multilocular cystic nephromas are benign tumors made up of cysts and are most common in infants, young children, and adult women. These tumors can occur in one or both kidneys. Children with this type of tumor also may have pleuropulmonary blastoma, so imaging tests that check the lungs for cysts or solid tumors are done. Since multilocular cystic nephroma may be an inherited condition, genetic counseling and genetic testing may be considered. See the PDQ summary about Unusual Cancers of Childhood Treatment for more information about pleuropulmonary blastoma. Primary Renal Synovial Sarcoma Primary renal synovial sarcoma is a cyst-like tumor of the kidney and is most common in young adults. These tumors grow and spread quickly. Anaplastic Sarcoma of the Kidney Anaplastic sarcoma of the kidney is a rare tumor that is most common in children or adolescents younger than 15 years of age. Anaplastic sarcoma of the kidney often spreads to the lungs, liver, or bones. Imaging tests that check the lungs for cysts or solid tumors may be done. Since anaplastic sarcoma may be an inherited condition, genetic counseling and genetic testing may be considered. Nephroblastomatosis is not cancer but may become Wilms tumor. Sometimes, after the kidneys form in the fetus, abnormal groups of kidney cells remain in one or both kidneys. In nephroblastomatosis (diffuse hyperplastic perilobar nephroblastomatosis), these abnormal groups of cells may grow in many places inside the kidney or make a thick layer around the kidney. When these groups of abnormal cells are found in a kidney after it was removed for Wilms tumor, the child has an increased risk of Wilms tumor in the other kidney. Frequent follow-up testing is important at least every 3 months, for at least 7 years after the child is treated. Treatment for Wilms tumor and other childhood kidney tumors may include genetic counseling. Genetic counseling (a discussion with a trained professional about genetic diseases and whether genetic testing is needed) may be needed if the child has one of the following syndromes or conditions: - A genetic syndrome or condition that increases the risk of Wilms tumor. - An inherited condition that increases the risk of renal cell cancer. - Rhabdoid tumor of the kidney. - Multilocular cystic nephroma.
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Wilms Tumor and Other Childhood Kidney Tumors
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susceptibility
Who is at risk for Wilms Tumor and Other Childhood Kidney Tumors? ?
Having certain genetic syndromes or other conditions can increase the risk of Wilms tumor. Anything that increases the risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk to your child's doctor if you think your child may be at risk. Wilms tumor may be part of a genetic syndrome that affects growth or development. A genetic syndrome is a set of signs and symptoms or conditions that occur together and is caused by certain changes in the genes. Certain conditions can also increase a child's risk of developing Wilms tumor. These and other genetic syndromes and conditions have been linked to Wilms tumor: - WAGR syndrome (Wilms tumor, aniridia, abnormal genitourinary system, and mental retardation). - Denys-Drash syndrome (abnormal genitourinary system). - Frasier syndrome (abnormal genitourinary system). - Beckwith-Wiedemann syndrome (abnormally large growth of one side of the body or a body part, large tongue, umbilical hernia at birth, and abnormal genitourinary system). - A family history of Wilms tumor. - Aniridia (the iris, the colored part of the eye, is missing). - Isolated hemihyperplasia (abnormally large growth of one side of the body or a body part). - Urinary tract problems such as cryptorchidism or hypospadias. Having certain conditions may increase the risk of renal cell cancer. Renal cell cancer may be related to the following conditions: - Von Hippel-Lindau disease (an inherited condition that causes abnormal growth of blood vessels). Children with Von Hippel-Lindau disease should be checked yearly for renal cell cancer with an ultrasound of the abdomen or an MRI (magnetic resonance imaging) beginning at age 8 to 11 years. - Tuberous sclerosis (an inherited disease marked by noncancerous fatty cysts in the kidney). - Familial renal cell cancer (an inherited condition that occurs when certain changes in the genes that cause kidney cancer are passed down from the parent to the child). - Renal medullary cancer (a rare kidney cancer that grows and spreads quickly). - Hereditary leiomyomatosis (an inherited disorder that increases the risk of having cancer of the kidney, skin, and uterus). Prior chemotherapy or radiation therapy for a childhood cancer, such as neuroblastoma, soft tissue sarcoma, leukemia, or Wilms tumor may also increase the risk of renal cell cancer. See the Second Cancers section in the PDQ summary about Late Effects of Treatment for Childhood Cancer for more information.
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symptoms
What are the symptoms of Wilms Tumor and Other Childhood Kidney Tumors ?
Signs of Wilms tumor and other childhood kidney tumors include a lump in the abdomen and blood in the urine. Sometimes childhood kidney tumors do not cause signs and symptoms and the parent finds a mass in the abdomen by chance or the mass is found during a well-child health check up. These and other signs and symptoms may be caused by kidney tumors or by other conditions. Check with your child's doctor if your child has any of the following: - A lump, swelling, or pain in the abdomen. - Blood in the urine. - High blood pressure (headache, feeling very tired, chest pain, or trouble seeing or breathing). - Hypercalcemia (loss of appetite, nausea and vomiting, weakness, or feeling very tired). - Fever for no known reason. - Loss of appetite. - Weight loss for no known reason. Wilms tumor that has spread to the lungs or liver may cause the following signs and symptoms: - Cough. - Blood in the sputum. - Trouble breathing. - Pain in the abdomen.
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exams and tests
How to diagnose Wilms Tumor and Other Childhood Kidney Tumors ?
Tests are used to screen for Wilms tumor. Screening tests are done in children with an increased risk of Wilms tumor. These tests may help find cancer early and decrease the chance of dying from cancer. In general, children with an increased risk of Wilms tumor should be screened for Wilms tumor every three months until they are at least 8 years old. An ultrasound test of the abdomen is usually used for screening. Small Wilms tumors may be found and removed before symptoms occur. Children with Beckwith-Wiedemann syndrome or hemihyperplasia are also screened for liver and adrenal tumors that are linked to these genetic syndromes. A test to check the alpha-fetoprotein (AFP) level in the blood and an ultrasound of the abdomen are done until the child is 4 years old. An ultrasound of the kidneys is done after the child is 4 years old. In children with certain gene changes, a different schedule for ultrasound of the abdomen may be used. Children with aniridia and a certain gene change are screened for Wilms tumor every three months until they are 8 years old. An ultrasound test of the abdomen is used for screening. Some children develop Wilms tumor in both kidneys. These often appear when Wilms tumor is first diagnosed, but Wilms tumor may also occur in the second kidney after the child is successfully treated for Wilms tumor in one kidney. Children with an increased risk of a second Wilms tumor in the other kidney should be screened for Wilms tumor every three months for up to eight years. An ultrasound test of the abdomen may be used for screening. Tests that examine the kidney and the blood are used to detect (find) and diagnose Wilms tumor and other childhood kidney tumors. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. This test is done to check how well the liver and kidneys are working. - Renal function test : A procedure in which blood or urine samples are checked to measure the amounts of certain substances released into the blood or urine by the kidneys. A higher or lower than normal amount of a substance can be a sign that the kidneys are not working as they should. - Urinalysis : A test to check the color of urine and its contents, such as sugar, protein, blood, and bacteria. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. An ultrasound of the abdomen is done to diagnose a kidney tumor. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, abdomen, and pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye is injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging) with gadolinium: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the abdomen. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - X-ray: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body, such as the chest and abdomen. - PET-CT scan : A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself. A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The decision of whether to do a biopsy is based on the following: - The size of the tumor. - The stage of the cancer. - Whether cancer is in one or both kidneys. - Whether imaging tests clearly show the cancer. - Whether the tumor can be removed by surgery. - Whether the patient is in a clinical trial. A biopsy may be done before any treatment is given, after chemotherapy to shrink the tumor, or after surgery to remove the tumor.
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outlook
What is the outlook for Wilms Tumor and Other Childhood Kidney Tumors ?
Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options for Wilms tumor depend on the following: - How different the tumor cells are from normal kidney cells when looked at under a microscope. - The stage of the cancer. - The type of tumor. - The age of the child. - Whether the tumor can be completely removed by surgery. - Whether there are certain changes in chromosomes or genes. - Whether the cancer has just been diagnosed or has recurred (come back). The prognosis for renal cell cancer depends on the following: - The stage of the cancer. - Whether the cancer has spread to the lymph nodes. The prognosis for rhabdoid tumor of the kidney depends on the following: - The age of the child at the time of diagnosis. - The stage of the cancer. - Whether the cancer has spread to the brain or spinal cord. The prognosis for clear cell sarcoma of the kidney depends on the following: - The age of the child at the time of diagnosis. - The stage of the cancer.
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stages
What are the stages of Wilms Tumor and Other Childhood Kidney Tumors ?
Key Points - Wilms tumors are staged during surgery and with imaging tests. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - In addition to the stages, Wilms tumors are described by their histology. - The following stages are used for both favorable histology and anaplastic Wilms tumors: - Stage I - Stage II - Stage III - Stage IV - Stage V - The treatment of other childhood kidney tumors depends on the tumor type. Wilms tumors are staged during surgery and with imaging tests. The process used to find out if cancer has spread outside of the kidney to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The doctor will use results of the diagnostic and staging tests to help find out the stage of the disease. The following tests may be done to see if cancer has spread to other places in the body: - Lymph node biopsy : A surgical procedure in which lymph nodes in the abdomen are removed and a sample of tissue is checked under a microscope for signs of cancer. This procedure is also called lymphadenectomy or lymph node dissection. - Liver function test : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign that the liver is not working as it should. - X-ray of the chest and bones: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body, such as the chest. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen, pelvis, chest, and brain, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye is injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET-CT scan : A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself. A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the abdomen, pelvis, and brain. This procedure is also called nuclear magnetic resonance imaging (NMRI). - Bone scan : A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. An ultrasound of the major heart vessels is done to stage Wilms tumor. - Cystoscopy : A procedure to look inside the bladder and urethra to check for abnormal areas. A cystoscope is inserted through the urethra into the bladder. A cystoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if Wilms tumor spreads to the lung, the cancer cells in the lung are actually Wilms tumor cells. The disease is metastatic Wilms tumor, not lung cancer. In addition to the stages, Wilms tumors are described by their histology. The histology (how the cells look under a microscope) of the tumor affects the prognosis and the treatment of Wilms tumor. The histology may be favorable or anaplastic (unfavorable). Tumors with a favorable histology have a better prognosis and respond better to chemotherapy than anaplastic tumors. Tumor cells that are anaplastic divide quickly and under a microscope do not look like the type of cells they came from. Anaplastic tumors are harder to treat with chemotherapy than other Wilms tumors at the same stage. The following stages are used for both favorable histology and anaplastic Wilms tumors: Stage I In stage I, the tumor was completely removed by surgery and all of the following are true: - Cancer was found only in the kidney and had not spread to blood vessels in the renal sinus (the part of the kidney where it joins the ureter) or to the lymph nodes. - The outer layer of the kidney did not break open. - The tumor did not break open. - A biopsy was not done before the tumor was removed. - No cancer cells were found at the edges of the area where the tumor was removed. Stage II In stage II, the tumor was completely removed by surgery and no cancer cells were found at the edges of the area where the cancer was removed. Cancer has not spread to lymph nodes. Before the tumor was removed, one of the following was true: - Cancer had spread to the renal sinus (the part of the kidney where it joins the ureter). - Cancer had spread to blood vessels outside the area of the kidney where urine is made, such as the renal sinus. Stage III In stage III, cancer remains in the abdomen after surgery and one of the following may be true: - Cancer has spread to lymph nodes in the abdomen or pelvis (the part of the body between the hips). - Cancer has spread to or through the surface of the peritoneum (the layer of tissue that lines the abdominal cavity and covers most organs in the abdomen). - A biopsy of the tumor was done before it was removed. - The tumor broke open before or during surgery to remove it. - The tumor was removed in more than one piece. - Cancer cells are found at the edges of the area where the tumor was removed. - The entire tumor could not be removed because important organs or tissues in the body would be damaged. Stage IV In stage IV, cancer has spread through the blood to organs such as the lungs, liver, bone, or brain, or to lymph nodes outside of the abdomen and pelvis. Stage V In stage V, cancer cells are found in both kidneys when the cancer is first diagnosed. The treatment of other childhood kidney tumors depends on the tumor type.
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treatment
What are the treatments for Wilms Tumor and Other Childhood Kidney Tumors ?
Key Points - There are different types of treatment for patients with Wilms tumor and other childhood kidney tumors. - Children with Wilms tumor or other childhood kidney tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children. - Some cancer treatments cause side effects months or years after treatment has ended. - Five types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - Biologic therapy - High-dose chemotherapy with stem cell rescue - New types of treatment are being tested in clinical trials. - Targeted therapy - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with Wilms tumor and other childhood kidney tumors. Different types of treatment are available for children with Wilms and other childhood kidney tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment. Children with Wilms tumor or other childhood kidney tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children. Your child's treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with Wilms tumor or other childhood kidney tumors and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Pediatric surgeon or urologist. - Radiation oncologist. - Rehabilitation specialist. - Pediatric nurse specialist. - Social worker. Some cancer treatments cause side effects months or years after treatment has ended. Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following: - Physical problems, such as heart problems, kidney problems, or problems during pregnancy. - Changes in mood, feelings, thinking, learning, or memory. - Second cancers (new types of cancer), such as cancer of the gastrointestinal tract or breast cancer. Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary about Late Effects of Treatment for Childhood Cancer for more information). Clinical trials are being done to find out if lower doses of chemotherapy and radiation can be used to lessen the late effects of treatment without changing how well the treatment works. Five types of standard treatment are used: Surgery Two types of surgery are used to treat kidney tumors: - Nephrectomy: Wilms tumor and other childhood kidney tumors are usually treated with nephrectomy (surgery to remove the whole kidney). Nearby lymph nodes may also be removed and checked for signs of cancer. Sometimes a kidney transplant (surgery to remove the kidney and replace it with a kidney from a donor) is done when the cancer is in both kidneys and the kidneys are not working well. - Partial nephrectomy: If cancer is found in both kidneys or is likely to spread to both kidneys, surgery may include a partial nephrectomy (removal of the cancer in the kidney and a small amount of normal tissue around it). Partial nephrectomy is done to keep as much of the kidney working as possible. A partial nephrectomy is also called renal-sparing surgery. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk of the cancer coming back, is called adjuvant therapy. Sometimes, a second-look surgery is done to see if cancer remains after chemotherapy or radiation therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated and whether a biopsy was done before surgery to remove the tumor. External radiation therapy is used to treat Wilms tumor and other childhood kidney tumors. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using two or more anticancer drugs. The way the chemotherapy is given depends on the type and stage of the cancer being treated. Systemic chemotherapy is used to treat Wilms tumor and other childhood kidney tumors. Sometimes the tumor cannot be removed by surgery for one of the following reasons: - The tumor is too close to important organs or blood vessels. - The tumor is too large to remove. - The cancer is in both kidneys. - There is a blood clot in the vessels near the liver. - The patient has trouble breathing because cancer has spread to the lungs. In this case, a biopsy is done first. Then chemotherapy is given to reduce the size of the tumor before surgery, in order to save as much healthy tissue as possible and lessen problems after surgery. This is called neoadjuvant chemotherapy. Radiation therapy is given after surgery. See Drugs Approved for Wilms Tumor and Other Childhood Kidney Cancers for more information. Biologic therapy Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon and interleukin-2 (IL-2) are types of biologic therapy used to treat childhood renal cell cancer. Interferon affects the division of cancer cells and can slow tumor growth. IL-2 boosts the growth and activity of many immune cells, especially lymphocytes (a type of white blood cell). Lymphocytes can attack and kill cancer cells. High-dose chemotherapy with stem cell rescue High-dose chemotherapy with stem cell rescue is a method of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These re-infused stem cells grow into (and restore) the body's blood cells. High-dose chemotherapy with stem cell rescue may be used to treat recurrent Wilms tumor. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Wilms Tumor Stage I Wilms Tumor Treatment of stage I Wilms tumor with favorable histology may include: - Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. - A clinical trial of nephrectomy only. Treatment of stage I anaplastic Wilms tumor may include: - Nephrectomy with removal of lymph nodes followed by combination chemotherapy and radiation therapy to the flank area (either side of the body between the ribs and hipbone). Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I Wilms tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Stage II Wilms Tumor Treatment of stage II Wilms tumor with favorable histology may include: - Nephrectomy with removal of lymph nodes, followed by combination chemotherapy. Treatment of stage II anaplastic Wilms tumor may include: - Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II Wilms tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Stage III Wilms Tumor Treatment of stage III Wilms tumor with favorable histology may include: - Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. Treatment of stage III anaplastic Wilms tumor may include: - Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. - Combination chemotherapy followed by nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III Wilms tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Stage IV Wilms Tumor Treatment of stage IV Wilms tumor with favorable histology may include: - Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. If cancer has spread to other parts of the body, patients will also receive radiation therapy to those areas. Treatment of stage IV anaplastic Wilms tumor may include: - Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. If cancer has spread to other parts of the body, patients will also receive radiation therapy to those areas. - Combination chemotherapy given before nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen. If cancer has spread to other parts of the body, patients will also receive radiation therapy to those areas. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV Wilms tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Stage V Wilms Tumor and patients at high risk of developing bilateral Wilms tumor Treatment of stage V Wilms tumor may be different for each patient and may include: - Combination chemotherapy to shrink the tumor, followed by repeat imaging at 4 to 8 weeks to decide on further therapy (partial nephrectomy, biopsy, continued chemotherapy, and/or radiation therapy). - A biopsy of the kidneys is followed by combination chemotherapy to shrink the tumor. A second surgery is done to remove as much of the cancer as possible. This may be followed by more chemotherapy and/or radiation therapy if cancer remains after surgery. If a kidney transplant is needed because of kidney problems, it is delayed until 1 to 2 years after treatment is completed and there are no signs of cancer. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage V Wilms tumor. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Treatment Options for Other Childhood Kidney Tumors Renal Cell Cancer (RCC) Treatment of renal cell cancer usually includes: - Surgery, which may be: - nephrectomy with removal of lymph nodes; or - partial nephrectomy with removal of lymph nodes. - Biologic therapy (interferon and interleukin-2) for cancer that has spread to other parts of the body. See the PDQ summary about Renal Cell Cancer Treatment for more information. Check the list of NCI-supported cancer clinical trials that are now accepting patients with renal cell carcinoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Rhabdoid Tumor of the Kidney There is no standard treatment for rhabdoid tumor of the kidney. Treatment may include: - A combination of surgery, chemotherapy, and/or radiation therapy. - A clinical trial of targeted therapy (tazemetostat). Check the list of NCI-supported cancer clinical trials that are now accepting patients with rhabdoid tumor of the kidney. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Clear Cell Sarcoma of the Kidney Treatment of clear cell sarcoma of the kidney may include: - Nephrectomy with removal of lymph nodes followed by combination chemotherapy and radiation therapy to the abdomen. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with clear cell sarcoma of the kidney. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Congenital Mesoblastic Nephroma Treatment for congenital mesoblastic nephroma usually includes: - Surgery that may be followed by chemotherapy. - A clinical trial of targeted therapy (LOXO-101 or entrectinib). Check the list of NCI-supported cancer clinical trials that are now accepting patients with congenital mesoblastic nephroma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Ewing Sarcoma of the Kidney There is no standard treatment for Ewing sarcoma of the kidney. Treatment may include: - A combination of surgery, chemotherapy, and radiation therapy. It may also be treated in the same way that Ewing sarcoma is treated. See the PDQ summary about Ewing Sarcoma Treatment for more information. Check the list of NCI-supported cancer clinical trials that are now accepting patients with Ewing sarcoma/peripheral primitive neuroectodermal tumor (PNET). For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website. Primary Renal Myoepithelial Carcinoma There is no standard treatment for primary renal myoepithelial carcinoma. Treatment may include: - A combination of surgery, chemotherapy, and radiation therapy. Cystic Partially Differentiated Nephroblastoma Treatment of cystic partially differentiated nephroblastoma may include: - Surgery that may be followed by chemotherapy. Multilocular Cystic Nephroma Treatment of multilocular cystic nephroma usually includes: - Surgery. Primary Renal Synovial Sarcoma Treatment of primary renal synovial sarcoma usually includes: - Chemotherapy. Anaplastic Sarcoma of the Kidney There is no standard treatment for anaplastic sarcoma of the kidney. Treatment is usually the same treatment given for anaplastic Wilms tumor. Nephroblastomatosis (Diffuse Hyperplastic Perilobar Nephroblastomatosis) The treatment of nephroblastomatosis depends on the following: - Whether the child has abnormal groups of cells in one or both kidneys. - Whether the child has Wilms tumor in one kidney and groups of abnormal cells in the other kidney. Treatment of nephroblastomatosis may include: - Chemotherapy followed by nephrectomy. Sometimes a partial nephrectomy may be done to keep as much kidney function as possible.
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research
what research (or clinical trials) is being done for Wilms Tumor and Other Childhood Kidney Tumors ?
New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Targeted therapy Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Targeted therapy used to treat childhood kidney tumors may include the following: - Monoclonal antibodies: This targeted therapy uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Lorvotuzumab is being studied in the treatment of recurrent Wilms tumor. - Kinase inhibitors: This targeted therapy blocks signals that cancer cells need to grow and divide. LOXO-101 and entrectinib are kinase inhibitors being studied to treat congenital mesoblastic nephroma. - Histone methyltransferase inhibitors: This targeted therapy slows down the cancer cell's ability to grow and divide. Tazemetostat is being studied in the treatment of rhabdoid tumor of the kidney. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
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Nasopharyngeal Cancer
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information
What is (are) Nasopharyngeal Cancer ?
Key Points - Nasopharyngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the nasopharynx. - Ethnic background and being exposed to the Epstein-Barr virus can affect the risk of nasopharyngeal cancer. - Signs of nasopharyngeal cancer include trouble breathing, speaking, or hearing. - Tests that examine the nose and throat are used to detect (find) and diagnose nasopharyngeal cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Nasopharyngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the nasopharynx. The nasopharynx is the upper part of the pharynx (throat) behind the nose. The pharynx is a hollow tube about 5 inches long that starts behind the nose and ends at the top of the trachea (windpipe) and esophagus (the tube that goes from the throat to the stomach). Air and food pass through the pharynx on the way to the trachea or the esophagus. The nostrils lead into the nasopharynx. An opening on each side of the nasopharynx leads into an ear. Nasopharyngeal cancer most commonly starts in the squamous cells that line the nasopharynx. Nasopharyngeal cancer is a type of head and neck cancer.
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Nasopharyngeal Cancer
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susceptibility
Who is at risk for Nasopharyngeal Cancer? ?
Ethnic background and being exposed to the Epstein-Barr virus can affect the risk of nasopharyngeal cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for nasopharyngeal cancer include the following: - Having Chinese or Asian ancestry. - Being exposed to the Epstein-Barr virus: The Epstein-Barr virus has been associated with certain cancers, including nasopharyngeal cancer and some lymphomas. - Drinking large amounts of alcohol.
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Nasopharyngeal Cancer
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symptoms
What are the symptoms of Nasopharyngeal Cancer ?
Signs of nasopharyngeal cancer include trouble breathing, speaking, or hearing. These and other signs and symptoms may be caused by nasopharyngeal cancer or by other conditions. Check with your doctor if you have any of the following: - A lump in the nose or neck. - A sore throat. - Trouble breathing or speaking. - Nosebleeds. - Trouble hearing. - Pain or ringing in the ear. - Headaches.
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Nasopharyngeal Cancer
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exams and tests
How to diagnose Nasopharyngeal Cancer ?
Tests that examine the nose and throat are used to detect (find) and diagnose nasopharyngeal cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as swollen lymph nodes in the neck or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a persons mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The tissue sample is removed during one of the following procedures: - Nasoscopy : A procedure to look inside the nose for abnormal areas. A nasoscope is inserted through the nose. A nasoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. - Upper endoscopy : A procedure to look at the inside of the nose, throat, esophagus, stomach, and duodenum (first part of the small intestine, near the stomach). An endoscope is inserted through the mouth and into the esophagus, stomach, and duodenum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples. The tissue samples are checked under a microscope for signs of cancer. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. PET scans may be used to find nasopharyngeal cancers that have spread to the bone. Sometimes a PET scan and a CT scan are done at the same time. If there is any cancer, this increases the chance that it will be found. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following: - The number of red blood cells, white blood cells, and platelets. - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - Epstein-Barr virus (EBV) test: A blood test to check for antibodies to the Epstein-Barr virus and DNA markers of the Epstein-Barr virus. These are found in the blood of patients who have been infected with EBV. - Hearing test: A procedure to check whether soft and loud sounds and low- and high-pitched sounds can be heard. Each ear is checked separately.
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Nasopharyngeal Cancer
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outlook
What is the outlook for Nasopharyngeal Cancer ?
Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer (whether it affects part of the nasopharynx, involves the whole nasopharynx, or has spread to other places in the body). - The type of nasopharyngeal cancer. - The size of the tumor. - The patients age and general health.
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Nasopharyngeal Cancer
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stages
What are the stages of Nasopharyngeal Cancer ?
Key Points - After nasopharyngeal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the nasopharynx or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for nasopharyngeal cancer: - Stage 0 (Carcinoma in Situ) - Stage I - Stage II - Stage III - Stage IV After nasopharyngeal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the nasopharynx or to other parts of the body. The process used to find out whether cancer has spread within the nasopharynx or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose nasopharyngeal cancer are often also used to stage the disease. (See the General Information section.) There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if nasopharyngeal cancer spreads to the lung, the cancer cells in the lung are actually nasopharyngeal cancer cells. The disease is metastatic nasopharyngeal cancer, not lung cancer. The following stages are used for nasopharyngeal cancer: Stage 0 (Carcinoma in Situ) In stage 0, abnormal cells are found in the lining of the nasopharynx. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. Stage I In stage I, cancer has formed and the cancer: - is found in the nasopharynx only; or - has spread from the nasopharynx to the oropharynx and/or to the nasal cavity. The oropharynx is the middle part of the throat and includes the soft palate, the base of the tongue, and the tonsils. Stage II In stage II nasopharyngeal cancer, the cancer: - is found in the nasopharynx only or has spread from the nasopharynx to the oropharynx and/or to the nasal cavity. Cancer has spread to one or more lymph nodes on one side of the neck and/or to lymph nodes behind the pharynx. The affected lymph nodes are 6 centimeters or smaller; or - is found in the parapharyngeal space. Cancer may have spread to one or more lymph nodes on one side of the neck and/or to lymph nodes behind the pharynx. The affected lymph nodes are 6 centimeters or smaller. The oropharynx is the middle part of the throat and includes the soft palate, the base of the tongue, and the tonsils. The parapharyngeal space is a fat-filled, triangular area near the pharynx, between the base of the skull and the lower jaw. Stage III In stage III nasopharyngeal cancer, the cancer: - is found in the nasopharynx only or has spread from the nasopharynx to the oropharynx and/or to the nasal cavity. Cancer has spread to one or more lymph nodes on both sides of the neck. The affected lymph nodes are 6 centimeters or smaller; or - is found in the parapharyngeal space. Cancer has spread to one or more lymph nodes on both sides of the neck. The affected lymph nodes are 6 centimeters or smaller; or - has spread to nearby bones or sinuses. Cancer may have spread to one or more lymph nodes on one or both sides of the neck and/or to lymph nodes behind the pharynx. The affected lymph nodes are 6 centimeters or smaller. The oropharynx is the middle part of the throat and includes the soft palate, the base of the tongue, and the tonsils. The parapharyngeal space is a fat-filled, triangular area near the pharynx, between the base of the skull and the lower jaw. Stage IV Stage IV nasopharyngeal cancer is divided into stages IVA, IVB, and IVC. - Stage IVA: Cancer has spread beyond the nasopharynx and may have spread to the cranial nerves, the hypopharynx (bottom part of the throat), areas in and around the side of the skull or jawbone, and/or the bone around the eye. Cancer may also have spread to one or more lymph nodes on one or both sides of the neck and/or to lymph nodes behind the pharynx. The affected lymph nodes are 6 centimeters or smaller. - Stage IVB: Cancer has spread to lymph nodes between the collarbone and the top of the shoulder and/or the affected lymph nodes are larger than 6 centimeters. - Stage IVC: Cancer has spread beyond nearby lymph nodes to other parts of the body.
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Nasopharyngeal Cancer
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treatment
What are the treatments for Nasopharyngeal Cancer ?
Key Points - There are different types of treatment for patients with nasopharyngeal cancer. - Three types of standard treatment are used: - Radiation therapy - Chemotherapy - Surgery - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with nasopharyngeal cancer. Different types of treatment are available for patients with nasopharyngeal cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Three types of standard treatment are used: Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following: - Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. Compared to standard radiation therapy, intensity-modulated radiation therapy may be less likely to cause dry mouth. - Stereotactic radiation therapy: A rigid head frame is attached to the skull to keep the head still during the radiation treatment. A machine aims radiation directly at the tumor. The total dose of radiation is divided into several smaller doses given over several days. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External and internal radiation therapy are used to treat nasopharyngeal cancer. External radiation therapy to the thyroid or the pituitary gland may change the way the thyroid gland works. A blood test to check the thyroid hormone level in the blood is done before and after therapy to make sure the thyroid gland is working properly. It is also important that a dentist check the patients teeth, gums, and mouth, and fix any existing problems before radiation therapy begins. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Chemotherapy may be given after radiation therapy to kill any cancer cells that are left. Treatment given after radiation therapy, to lower the risk that the cancer will come back, is called adjuvant therapy. See Drugs Approved for Head and Neck Cancer for more information. (Nasopharyngeal cancer is a type of head and neck cancer.) Surgery Surgery is a procedure to find out whether cancer is present, to remove cancer from the body, or to repair a body part. Also called an operation. Surgery is sometimes used for nasopharyngeal cancer that does not respond to radiation therapy. If cancer has spread to the lymph nodes, the doctor may remove lymph nodes and other tissues in the neck. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage I Nasopharyngeal Cancer Treatment of stage I nasopharyngeal cancer is usually radiation therapy to the tumor and lymph nodes in the neck. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I nasopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Nasopharyngeal Cancer Treatment of stage II nasopharyngeal cancer may include the following: - Chemotherapy given with radiation therapy, followed by more chemotherapy. - Radiation therapy to the tumor and lymph nodes in the neck. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II nasopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Nasopharyngeal Cancer Treatment of stage III nasopharyngeal cancer may include the following: - Chemotherapy given with radiation therapy, which may be followed by more chemotherapy. - Radiation therapy. - Radiation therapy followed by surgery to remove cancer -containing lymph nodes in the neck that remain or come back after radiation therapy. - A clinical trial of chemotherapy given before, with, or after radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III nasopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Nasopharyngeal Cancer Treatment of stage IV nasopharyngeal cancer may include the following: - Chemotherapy given with radiation therapy, followed by more chemotherapy. - Radiation therapy. - Radiation therapy followed by surgery to remove cancer -containing lymph nodes in the neck that remain or come back after radiation therapy. - Chemotherapy for cancer that has metastasized (spread) to other parts of the body. - A clinical trial of chemotherapy given before, with, or after radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV nasopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
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Nasopharyngeal Cancer
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research
what research (or clinical trials) is being done for Nasopharyngeal Cancer ?
New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
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Salivary Gland Cancer
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information
What is (are) Salivary Gland Cancer ?
Key Points - Salivary gland cancer is a rare disease in which malignant (cancer) cells form in the tissues of the salivary glands. - Being exposed to certain types of radiation may increase the risk of salivary cancer. - Signs of salivary gland cancer include a lump or trouble swallowing. - Tests that examine the head, neck, and the inside of the mouth are used to detect (find) and diagnose salivary gland cancer. - Certain factors affect treatment options and prognosis (chance of recovery). Salivary gland cancer is a rare disease in which malignant (cancer) cells form in the tissues of the salivary glands. The salivary glands make saliva and release it into the mouth. Saliva has enzymes that help digest food and antibodies that help protect against infections of the mouth and throat. There are 3 pairs of major salivary glands: - Parotid glands: These are the largest salivary glands and are found in front of and just below each ear. Most major salivary gland tumors begin in this gland. - Sublingual glands: These glands are found under the tongue in the floor of the mouth. - Submandibular glands: These glands are found below the jawbone. There are also hundreds of small (minor) salivary glands lining parts of the mouth, nose, and larynx that can be seen only with a microscope. Most small salivary gland tumors begin in the palate (roof of the mouth). More than half of all salivary gland tumors are benign (not cancerous) and do not spread to other tissues. Salivary gland cancer is a type of head and neck cancer.
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Salivary Gland Cancer
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susceptibility
Who is at risk for Salivary Gland Cancer? ?
Being exposed to certain types of radiation may increase the risk of salivary cancer. Anything that increases the chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesnt mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Although the cause of most salivary gland cancers is not known, risk factors include the following: - Older age. - Treatment with radiation therapy to the head and neck. - Being exposed to certain substances at work.
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Salivary Gland Cancer
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symptoms
What are the symptoms of Salivary Gland Cancer ?
Signs of salivary gland cancer include a lump or trouble swallowing. Salivary gland cancer may not cause any symptoms. It may be found during a regular dental check-up or physical exam. Signs and symptoms may be caused by salivary gland cancer or by other conditions. Check with your doctor if you have any of the following: - A lump (usually painless) in the area of the ear, cheek, jaw, lip, or inside the mouth. - Fluid draining from the ear. - Trouble swallowing or opening the mouth widely. - Numbness or weakness in the face. - Pain in the face that does not go away.
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Salivary Gland Cancer
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exams and tests
How to diagnose Salivary Gland Cancer ?
Tests that examine the head, neck, and the inside of the mouth are used to detect (find) and diagnose salivary gland cancer. The following procedures may be used: - Physical exam and history : An exam of the body to check general signs of health. The head, neck, mouth, and throat will be checked for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Endoscopy : A procedure to look at organs and tissues inside the body to check for abnormal areas. For salivary gland cancer, an endoscope is inserted into the mouth to look at the mouth, throat, and larynx. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. - Fine needle aspiration (FNA) biopsy : The removal of tissue or fluid using a thin needle. An FNA is the most common type of biopsy used for salivary gland cancer. - Incisional biopsy : The removal of part of a lump or a sample of tissue that doesnt look normal. - Surgery : If cancer cannot be diagnosed from the sample of tissue removed during an FNA biopsy or an incisional biopsy, the mass may be removed and checked for signs of cancer. Because salivary gland cancer can be hard to diagnose, patients should ask to have the tissue samples checked by a pathologist who has experience in diagnosing salivary gland cancer.
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Salivary Gland Cancer
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outlook
What is the outlook for Salivary Gland Cancer ?
Certain factors affect treatment options and prognosis (chance of recovery). The treatment options and prognosis (chance of recovery) depend on the following: - The stage of the cancer (especially the size of the tumor). - The type of salivary gland the cancer is in. - The type of cancer cells (how they look under a microscope). - The patient's age and general health.
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Salivary Gland Cancer
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stages
What are the stages of Salivary Gland Cancer ?
Key Points - After salivary gland cancer has been diagnosed, tests are done to find out if cancer cells have spread within the salivary gland or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - The following stages are used for major salivary gland cancers: - Stage I - Stage II - Stage III - Stage IV After salivary gland cancer has been diagnosed, tests are done to find out if cancer cells have spread within the salivary gland or to other parts of the body. The process used to find out if cancer has spread within the salivary glands or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedures may be used in the staging process: - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if salivary gland cancer spreads to the lung, the cancer cells in the lung are actually salivary gland cancer cells. The disease is metastatic salivary gland cancer, not lung cancer. The following stages are used for major salivary gland cancers: Stage I In stage I, the tumor is in the salivary gland only and is 2 centimeters or smaller. Stage II In stage II, the tumor is in the salivary gland only and is larger than 2 centimeters but not larger than 4 centimeters. Stage III In stage III, one of the following is true: - The tumor is not larger than 4 centimeters and has spread to a single lymph node on the same side as the tumor and the lymph node is 3 centimeters or smaller. - The tumor is larger than 4 centimeters and/or has spread to soft tissue around the affected gland. Cancer may have spread to a single lymph node on the same side as the tumor and the lymph node is 3 centimeters or smaller. Stage IV Stage IV is divided into stages IVA, IVB, and IVC as follows: - Stage IVA: - The tumor may be any size and may have spread to soft tissue around the affected gland. Cancer has spread to a single lymph node on the same side as the tumor and the lymph node is larger than 3 centimeters but not larger than 6 centimeters, or has spread to more than one lymph node on either or both sides of the body and the lymph nodes are not larger than 6 centimeters; or - Cancer has spread to the skin, jawbone, ear canal, and/or facial nerve, and may have spread to one or more lymph nodes on either or both sides of the body. The lymph nodes are not larger than 6 centimeters. - Stage IVB: - The tumor may be any size and may have spread to soft tissue around the affected gland. Cancer has spread to a lymph node larger than 6 centimeters; or - Cancer has spread to the base of the skull and/or the carotid artery, and may have spread to one or more lymph nodes of any size on either or both sides of the body. - Stage IVC: - The tumor may be any size and may have spread to soft tissue around the affected gland, to the skin, jawbone, ear canal, facial nerve, base of the skull, or carotid artery, or to one or more lymph nodes on either or both sides of the body. Cancer has spread to distant parts of the body. Salivary gland cancers are also grouped by grade. The grade of a tumor tells how fast the cancer cells are growing, based on how the cells look under a microscope. Low-grade cancers grow more slowly than high-grade cancers. Minor salivary gland cancers are staged according to where they were first found in the body.
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Salivary Gland Cancer
7
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treatment
What are the treatments for Salivary Gland Cancer ?
Key Points - There are different types of treatment for patients with salivary gland cancer. - Patients with salivary gland cancer should have their treatment planned by a team of doctors who are experts in treating head and neck cancer. - Three types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - New types of treatment are being tested in clinical trials. - Radiosensitizers - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with salivary gland cancer. Different types of treatment are available for patients with salivary gland cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Patients with salivary gland cancer should have their treatment planned by a team of doctors who are experts in treating head and neck cancer. Your treatment will be overseen by a medical oncologist, a doctor who specializes in treating people with cancer. Because the salivary glands help in eating and digesting food, patients may need special help adjusting to the side effects of the cancer and its treatment. The medical oncologist may refer you to other doctors who have experience and expertise in treating patients with head and neck cancer and who specialize in certain areas of medicine. These include the following: - Head and neck surgeon. - Radiation oncologist. - Dentist. - Speech therapist. - Dietitian. - Psychologist. - Rehabilitation specialist. - Plastic surgeon. Three types of standard treatment are used: Surgery Surgery (removing the cancer in an operation) is a common treatment for salivary gland cancer. A doctor may remove the cancer and some of the healthy tissue around the cancer. In some cases, a lymphadenectomy (surgery in which lymph nodes are removed) will also be done. Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given radiation therapy after surgery to kill any cancer cells that are left. Treatment given after surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. Special types of external radiation may be used to treat some salivary gland tumors. These include: - Fast neutron radiation therapy: Fast neutron radiation therapy is a type of high-energy external radiation therapy. A radiation therapy machine aims neutrons (tiny, invisible particles) at the cancer cells to kill them. Fast neutron radiation therapy uses a higher-energy radiation than the x-ray type of radiation therapy. This allows the radiation therapy to be given in fewer treatments. - Photon-beam radiation therapy: Photon-beam radiation therapy is a type of external radiation therapy that reaches deep tumors with high-energy x-rays made by a machine called a linear accelerator. This can be delivered as hyperfractionated radiation therapy, in which the total dose of radiation is divided into small doses and the treatments are given more than once a day. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat salivary gland cancer, and may also be used as palliative therapy to relieve symptoms and improve quality of life. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. See Drugs Approved for Head and Neck Cancer for more information. (Salivary gland cancer is a type of head and neck cancer.) New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiosensitizers Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options by Stage Stage I Salivary Gland Cancer Treatment for stage I salivary gland cancer depends on whether the cancer is low-grade (slow growing) or high-grade (fast growing). If the cancer is low-grade, treatment may include the following: - Surgery with or without radiation therapy. - Fast neutron radiation therapy. If the cancer is high-grade, treatment may include the following: - Surgery with or without radiation therapy. - A clinical trial of chemotherapy. - A clinical trial of a new local therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I salivary gland cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage II Salivary Gland Cancer Treatment for stage II salivary gland cancer depends on whether the cancer is low-grade (slow growing) or high-grade (fast growing). If the cancer is low-grade, treatment may include the following: - Surgery with or without radiation therapy. - Radiation therapy. - Chemotherapy. If the cancer is high-grade, treatment may include the following: - Surgery with or without radiation therapy. - Fast neutron or photon-beam radiation therapy. - A clinical trial of radiation therapy and/or radiosensitizers. - A clinical trial of chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage II salivary gland cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage III Salivary Gland Cancer Treatment for stage III salivary gland cancer depends on whether the cancer is low-grade (slow growing) or high-grade (fast growing). If the cancer is low-grade, treatment may include the following: - Surgery with or without lymphadenectomy. Radiation therapy may also be given after surgery. - Radiation therapy. - Fast neutron radiation therapy to lymph nodes with cancer. - Chemotherapy. - A clinical trial of fast neutron radiation therapy to the tumor. - A clinical trial of chemotherapy. If the cancer is high-grade, treatment may include the following: - Surgery with or without lymphadenectomy. Radiation therapy may also be given after surgery. - Fast neutron radiation therapy. - Radiation therapy as palliative therapy to relieve symptoms and improve quality of life. - A clinical trial of radiation therapy and/or radiosensitizers. - A clinical trial of chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III salivary gland cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Stage IV Salivary Gland Cancer Treatment of stage IV salivary gland cancer may include the following: - Fast neutron or photon-beam radiation therapy. - A clinical trial of chemotherapy with or without radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage IV salivary gland cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
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Salivary Gland Cancer
8
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research
what research (or clinical trials) is being done for Salivary Gland Cancer ?
New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Radiosensitizers Radiosensitizers are drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor cells. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
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Bile Duct Cancer (Cholangiocarcinoma)
1
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information
What is (are) Bile Duct Cancer (Cholangiocarcinoma) ?
Key Points - Bile duct cancer is a rare disease in which malignant (cancer) cells form in the bile ducts. - Having colitis or certain liver diseases can increase the risk of bile duct cancer. - Signs of bile duct cancer include jaundice and pain in the abdomen. - Tests that examine the bile ducts and nearby organs are used to detect (find), diagnose, and stage bile duct cancer. - Different procedures may be used to obtain a sample of tissue and diagnose bile duct cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Bile duct cancer is a rare disease in which malignant (cancer) cells form in the bile ducts. A network of tubes, called ducts, connects the liver, gallbladder, and small intestine. This network begins in the liver where many small ducts collect bile (a fluid made by the liver to break down fats during digestion). The small ducts come together to form the right and left hepatic ducts, which lead out of the liver. The two ducts join outside the liver and form the common hepatic duct. The cystic duct connects the gallbladder to the common hepatic duct. Bile from the liver passes through the hepatic ducts, common hepatic duct, and cystic duct and is stored in the gallbladder. When food is being digested, bile stored in the gallbladder is released and passes through the cystic duct to the common bile duct and into the small intestine. Bile duct cancer is also called cholangiocarcinoma. There are two types of bile duct cancer: - Intrahepatic bile duct cancer : This type of cancer forms in the bile ducts inside the liver. Only a small number of bile duct cancers are intrahepatic. Intrahepatic bile duct cancers are also called intrahepatic cholangiocarcinomas. - Extrahepatic bile duct cancer : The extrahepatic bile duct is made up of the hilum region and the distal region. Cancer can form in either region: - Perihilar bile duct cancer: This type of cancer is found in the hilum region, the area where the right and left bile ducts exit the liver and join to form the common hepatic duct. Perihilar bile duct cancer is also called a Klatskin tumor or perihilar cholangiocarcinoma. - Distal extrahepatic bile duct cancer: This type of cancer is found in the distal region. The distal region is made up of the common bile duct which passes through the pancreas and ends in the small intestine. Distal extrahepatic bile duct cancer is also called extrahepatic cholangiocarcinoma.
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Bile Duct Cancer (Cholangiocarcinoma)
2
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susceptibility
Who is at risk for Bile Duct Cancer (Cholangiocarcinoma)? ?
Having colitis or certain liver diseases can increase the risk of bile duct cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Risk factors for bile duct cancer include the following conditions: - Primary sclerosing cholangitis (a progressive disease in which the bile ducts become blocked by inflammation and scarring). - Chronic ulcerative colitis. - Cysts in the bile ducts (cysts block the flow of bile and can cause swollen bile ducts, inflammation, and infection). - Infection with a Chinese liver fluke parasite.
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Bile Duct Cancer (Cholangiocarcinoma)
3
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symptoms
What are the symptoms of Bile Duct Cancer (Cholangiocarcinoma) ?
Signs of bile duct cancer include jaundice and pain in the abdomen. These and other signs and symptoms may be caused by bile duct cancer or by other conditions. Check with your doctor if you have any of the following: - Jaundice (yellowing of the skin or whites of the eyes). - Dark urine. - Clay colored stool. - Pain in the abdomen. - Fever. - Itchy skin. - Nausea and vomiting. - Weight loss for an unknown reason.
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Bile Duct Cancer (Cholangiocarcinoma)
4
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exams and tests
How to diagnose Bile Duct Cancer (Cholangiocarcinoma) ?
Tests that examine the bile ducts and nearby organs are used to detect (find), diagnose, and stage bile duct cancer. Procedures that make pictures of the bile ducts and the nearby area help diagnose bile duct cancer and show how far the cancer has spread. The process used to find out if cancer cells have spread within and around the bile ducts or to distant parts of the body is called staging. In order to plan treatment, it is important to know if the bile duct cancer can be removed by surgery. Tests and procedures to detect, diagnose, and stage bile duct cancer are usually done at the same time. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Liver function tests : A procedure in which a blood sample is checked to measure the amounts of bilirubin and alkaline phosphatase released into the blood by the liver. A higher than normal amount of these substances can be a sign of liver disease that may be caused by bile duct cancer. - Laboratory tests : Medical procedures that test samples of tissue, blood, urine, or other substances in the body. These tests help to diagnose disease, plan and check treatment, or monitor the disease over time. - Carcinoembryonic antigen (CEA) and CA 19-9 tumor marker test : A procedure in which a sample of blood, urine, or tissue is checked to measure the amounts of certain substances made by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the body. These are called tumor markers. Higher than normal levels of carcinoembryonic antigen (CEA) and CA 19-9 may mean there is bile duct cancer. - Ultrasound exam : A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs, such as the abdomen, and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - MRCP (magnetic resonance cholangiopancreatography): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body such as the liver, bile ducts, gallbladder, pancreas, and pancreatic duct. Different procedures may be used to obtain a sample of tissue and diagnose bile duct cancer. Cells and tissues are removed during a biopsy so they can be viewed under a microscope by a pathologist to check for signs of cancer. Different procedures may be used to obtain the sample of cells and tissue. The type of procedure used depends on whether the patient is well enough to have surgery. Types of biopsy procedures include the following: - Laparoscopy : A surgical procedure to look at the organs inside the abdomen, such as the bile ducts and liver, to check for signs of cancer. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as taking tissue samples to be checked for signs of cancer. - Percutaneous transhepatic cholangiography (PTC): A procedure used to x-ray the liver and bile ducts. A thin needle is inserted through the skin below the ribs and into the liver. Dye is injected into the liver or bile ducts and an x-ray is taken. A sample of tissue is removed and checked for signs of cancer. If the bile duct is blocked, a thin, flexible tube called a stent may be left in the liver to drain bile into the small intestine or a collection bag outside the body. This procedure may be used when a patient cannot have surgery. - Endoscopic retrograde cholangiopancreatography (ERCP): A procedure used to x-ray the ducts (tubes) that carry bile from the liver to the gallbladder and from the gallbladder to the small intestine. Sometimes bile duct cancer causes these ducts to narrow and block or slow the flow of bile, causing jaundice. An endoscope is passed through the mouth and stomach and into the small intestine. Dye is injected through the endoscope (thin, tube-like instrument with a light and a lens for viewing) into the bile ducts and an x-ray is taken. A sample of tissue is removed and checked for signs of cancer. If the bile duct is blocked, a thin tube may be inserted into the duct to unblock it. This tube (or stent) may be left in place to keep the duct open. This procedure may be used when a patient cannot have surgery.
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Bile Duct Cancer (Cholangiocarcinoma)
5
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outlook
What is the outlook for Bile Duct Cancer (Cholangiocarcinoma) ?
Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - Whether the cancer is in the upper or lower part of the bile duct system. - The stage of the cancer (whether it affects only the bile ducts or has spread to the liver, lymph nodes, or other places in the body). - Whether the cancer has spread to nearby nerves or veins. - Whether the cancer can be completely removed by surgery. - Whether the patient has other conditions, such as primary sclerosing cholangitis. - Whether the level of CA 19-9 is higher than normal. - Whether the cancer has just been diagnosed or has recurred (come back). Treatment options may also depend on the symptoms caused by the cancer. Bile duct cancer is usually found after it has spread and can rarely be completely removed by surgery. Palliative therapy may relieve symptoms and improve the patient's quality of life.
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Bile Duct Cancer (Cholangiocarcinoma)
6
0000007_2-6
research
what research (or clinical trials) is being done for Bile Duct Cancer (Cholangiocarcinoma) ?
New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Liver transplant In a liver transplant, the entire liver is removed and replaced with a healthy donated liver. A liver transplant may be done in patients with perihilar bile duct cancer. If the patient has to wait for a donated liver, other treatment is given as needed. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
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Bile Duct Cancer (Cholangiocarcinoma)
7
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stages
What are the stages of Bile Duct Cancer (Cholangiocarcinoma) ?
Key Points - The results of diagnostic and staging tests are used to find out if cancer cells have spread. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - Stages are used to describe the different types of bile duct cancer. - Intrahepatic bile duct cancer - Perihilar bile duct cancer - Distal extrahepatic bile duct cancer - The following groups are used to plan treatment: - Resectable (localized) bile duct cancer - Unresectable, metastatic, or recurrent bile duct cancer The results of diagnostic and staging tests are used to find out if cancer cells have spread. The process used to find out if cancer has spread to other parts of the body is called staging. For bile duct cancer, the information gathered from tests and procedures is used to plan treatment, including whether the tumor can be removed by surgery. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if bile duct cancer spreads to the liver, the cancer cells in the liver are actually bile duct cancer cells. The disease is metastatic bile duct cancer, not liver cancer. Stages are used to describe the different types of bile duct cancer. Intrahepatic bile duct cancer - Stage 0: Abnormal cells are found in the innermost layer of tissue lining the intrahepatic bile duct. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. - Stage I: There is one tumor that has spread into the intrahepatic bile duct and it has not spread into any blood vessels. - Stage II: There is one tumor that has spread through the wall of the bile duct and into a blood vessel, or there are multiple tumors that may have spread into a blood vessel. - Stage III: The tumor has spread through the tissue that lines the abdominal wall or has spread to organs or tissues near the liver such as the duodenum, colon, and stomach. - Stage IV: Stage IV is divided into stage IVA and stage IVB. - Stage IVA: The cancer has spread along the outside of the intrahepatic bile ducts or the cancer has spread to nearby lymph nodes. - Stage IVB: The cancer has spread to organs in other parts of the body. Perihilar bile duct cancer - Stage 0: Abnormal cells are found in the innermost layer of tissue lining the perihilar bile duct. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. - Stage I: Cancer has formed in the innermost layer of the wall of the perihilar bile duct and has spread into the muscle layer or fibrous tissue layer of the wall. - Stage II: Cancer has spread through the wall of the perihilar bile duct to nearby fatty tissue or to the liver. - Stage III: Stage III is divided into stage IIIA and stage IIIB. - Stage IIIA: Cancer has spread to branches on one side of the hepatic artery or of the portal vein. - Stage IIIB: Cancer has spread to nearby lymph nodes. Cancer may have spread into the wall of the perihilar bile duct or through the wall to nearby fatty tissue, the liver, or to branches on one side of the hepatic artery or of the portal vein. - Stage IV: Stage IV is divided into stage IVA and stage IVB. - Stage IVA: Cancer has spread to one or more of the following: - the main part of the portal vein and/or common hepatic artery; - the branches of the portal vein and/or common hepatic artery on both sides; - the right hepatic duct and the left branch of the hepatic artery or of the portal vein; - the left hepatic duct and the right branch of the hepatic artery or of the portal vein. Cancer may have spread to nearby lymph nodes. - Stage IVB: Cancer has spread to lymph nodes in more distant parts of the abdomen, or to organs in other parts of the body. Distal extrahepatic bile duct cancer - Stage 0: Abnormal cells are found in the innermost layer of tissue lining the distal extrahepatic bile duct. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ. - Stage I: Stage I is divided into stage IA and stage IB. - Stage IA: Cancer has formed and is found in the distal extrahepatic bile duct wall only. - Stage IB: Cancer has formed and has spread through the wall of the distal extrahepatic bile duct but has not spread to nearby organs. - Stage II: Stage II is divided into stage IIA and stage IIB. - Stage IIA: Cancer has spread from the distal extrahepatic bile duct to the gallbladder, pancreas, duodenum, or other nearby organs. - Stage IIB: Cancer has spread from the distal extrahepatic bile duct to nearby lymph nodes. Cancer may have spread through the wall of the duct or to nearby organs. - Stage III: Cancer has spread to the large vessels that carry blood to the organs in the abdomen. Cancer may have spread to nearby lymph nodes. - Stage IV: Cancer has spread to organs in distant parts of the body. The following groups are used to plan treatment: Resectable (localized) bile duct cancer The cancer is in an area, such as the lower part of the common bile duct or perihilar area, where it can be removed completely by surgery. Unresectable, metastatic, or recurrent bile duct cancer Unresectable cancer cannot be removed completely by surgery. Most patients with bile duct cancer cannot have their cancer completely removed by surgery. Metastasis is the spread of cancer from the primary site (place where it started) to other places in the body. Metastatic bile duct cancer may have spread to the liver, other parts of the abdominal cavity, or to distant parts of the body. Recurrent bile duct cancer is cancer that has recurred (come back) after it has been treated. The cancer may come back in the bile ducts, liver, or gallbladder. Less often, it may come back in distant parts of the body.
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Bile Duct Cancer (Cholangiocarcinoma)
8
0000007_2-8
treatment
What are the treatments for Bile Duct Cancer (Cholangiocarcinoma) ?
Key Points - There are different types of treatment for patients with bile duct cancer. - Three types of standard treatment are used: - Surgery - Radiation therapy - Chemotherapy - New types of treatment are being tested in clinical trials. - Liver transplant - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their cancer treatment. - Follow-up tests may be needed. There are different types of treatment for patients with bile duct cancer. Different types of treatments are available for patients with bile duct cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment. Three types of standard treatment are used: Surgery The following types of surgery are used to treat bile duct cancer: - Removal of the bile duct: A surgical procedure to remove part of the bile duct if the tumor is small and in the bile duct only. Lymph nodes are removed and tissue from the lymph nodes is viewed under a microscope to see if there is cancer. - Partial hepatectomy: A surgical procedure in which the part of the liver where cancer is found is removed. The part removed may be a wedge of tissue, an entire lobe, or a larger part of the liver, along with some normal tissue around it. - Whipple procedure: A surgical procedure in which the head of the pancreas, the gallbladder, part of the stomach, part of the small intestine, and the bile duct are removed. Enough of the pancreas is left to make digestive juices and insulin. Even if the doctor removes all the cancer that can be seen at the time of the operation, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. It is not yet known whether chemotherapy or radiation therapy given after surgery helps keep the cancer from coming back. The following types of palliative surgery may be done to relieve symptoms caused by a blocked bile duct and improve quality of life: - Biliary bypass: A surgical procedure in which the part of the bile duct before the blockage is connected with part of the bile duct that is past the blockage or to the small intestine. This allows bile to flow to the gallbladder or small intestine. - Stent placement: A surgical procedure in which a stent (a thin, flexible tube or metal tube) is placed in the bile duct to open it and allow bile to flow into the small intestine or through a catheter that goes to a collection bag outside of the body. - Percutaneous transhepatic biliary drainage: A procedure used to x-ray the liver and bile ducts. A thin needle is inserted through the skin below the ribs and into the liver. Dye is injected into the liver or bile ducts and an x-ray is taken. If the bile duct is blocked, a thin, flexible tube called a stent may be left in the liver to drain bile into the small intestine or a collection bag outside the body. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy: - External radiation therapy uses a machine outside the body to send radiation toward the cancer. - Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. External and internal radiation therapy are used to treat bile duct cancer. It is not yet known whether external radiation therapy helps in the treatment of resectable bile duct cancer. In unresectable, metastatic, or recurrent bile duct cancer, new ways to improve the effect of external radiation therapy on cancer cells are being studied: - Hyperthermia therapy: A treatment in which body tissue is exposed to high temperatures to make cancer cells more sensitive to the effects of radiation therapy and certain anticancer drugs. - Radiosensitizers: Drugs that make cancer cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more cancer cells. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Systemic chemotherapy is used to treat unresectable, metastatic, or recurrent bile duct cancer. It is not yet known whether systemic chemotherapy helps in the treatment of resectable bile duct cancer. In unresectable, metastatic, or recurrent bile duct cancer, intra-arterial embolization is being studied. It is a procedure in which the blood supply to a tumor is blocked after anticancer drugs are given in blood vessels near the tumor. Sometimes, the anticancer drugs are attached to small beads that are injected into an artery that feeds the tumor. The beads block blood flow to the tumor as they release the drug. This allows a higher amount of drug to reach the tumor for a longer period of time, which may kill more cancer cells. New types of treatment are being tested in clinical trials. This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website. Liver transplant In a liver transplant, the entire liver is removed and replaced with a healthy donated liver. A liver transplant may be done in patients with perihilar bile duct cancer. If the patient has to wait for a donated liver, other treatment is given as needed. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their cancer treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials. Follow-up tests may be needed. Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for Bile Duct Cancer Intrahepatic Bile Duct Cancer Resectable Intrahepatic Bile Duct Cancer Treatment of resectable intrahepatic bile duct cancer may include: - Surgery to remove the cancer, which may include partial hepatectomy. Embolization may be done before surgery. - Surgery followed by chemotherapy and/or radiation therapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I intrahepatic bile duct cancer and stage II intrahepatic bile duct cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Unresectable, Recurrent, or Metastatic Intrahepatic Bile Duct Cancer Treatment of unresectable, recurrent, or metastatic intrahepatic bile duct cancer may include the following: - Stent placement as palliative treatment to relieve symptoms and improve quality of life. - External or internal radiation therapy as palliative treatment to relieve symptoms and improve the quality of life. - Chemotherapy. - A clinical trial of external radiation therapy combined with hyperthermia therapy, radiosensitizer drugs, or chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III intrahepatic bile duct cancer, stage IV intrahepatic bile duct cancer and recurrent intrahepatic bile duct cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Perihilar Bile Duct Cancer Resectable Perihilar Bile Duct Cancer Treatment of resectable perihilar bile duct cancer may include the following: - Surgery to remove the cancer, which may include partial hepatectomy. - Stent placement or percutaneous transhepatic biliary drainage as palliative therapy, to relieve jaundice and other symptoms and improve the quality of life. - Surgery followed by radiation therapy and/or chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage I perihilar bile duct cancer and stage II perihilar bile duct cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Unresectable, Recurrent, or Metastatic Perihilar Bile Duct Cancer Treatment of unresectable, recurrent, or metastatic perihilar bile duct cancer may include the following: - Stent placement or biliary bypass as palliative treatment to relieve symptoms and improve the quality of life. - External or internal radiation therapy as palliative treatment to relieve symptoms and improve the quality of life. - Chemotherapy. - A clinical trial of external radiation therapy combined with hyperthermia therapy, radiosensitizer drugs, or chemotherapy. - A clinical trial of chemotherapy and radiation therapy followed by a liver transplant. Check the list of NCI-supported cancer clinical trials that are now accepting patients with stage III perihilar bile duct cancer, stage IV perihilar bile duct cancer and recurrent perihilar bile duct cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Distal Extrahepatic Bile Duct Cancer Resectable Distal Extrahepatic Bile Duct Cancer Treatment of resectable distal extrahepatic bile duct cancer may include the following: - Surgery to remove the cancer, which may include a Whipple procedure. - Stent placement or percutaneous transhepatic biliary drainage as palliative therapy, to relieve jaundice and other symptoms and improve the quality of life. - Surgery followed by radiation therapy and/or chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with localized extrahepatic bile duct cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website. Unresectable, Recurrent, or Metastatic Distal Extrahepatic Bile Duct Cancer Treatment of unresectable, recurrent, or metastatic distal extrahepatic bile duct cancer may include the following: - Stent placement or biliary bypass as palliative treatment to relieve symptoms and improve the quality of life. - External or internal radiation therapy as palliative treatment to relieve symptoms and improve the quality of life. - Chemotherapy. - A clinical trial of external radiation therapy combined with hyperthermia therapy, radiosensitizer drugs, or chemotherapy. Check the list of NCI-supported cancer clinical trials that are now accepting patients with unresectable extrahepatic bile duct cancer, recurrent extrahepatic bile duct cancer and metastatic extrahepatic bile duct cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
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Neuroblastoma
1
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information
What is (are) Neuroblastoma ?
Key Points - Neuroblastoma is a disease in which malignant (cancer) cells form in nerve tissue. - Most cases of neuroblastoma are diagnosed before 1 year of age. - The risk factors for neuroblastoma are not known. Neuroblastoma is a disease in which malignant (cancer) cells form in nerve tissue. Neuroblastoma often begins in the nerve tissue of the adrenal glands. There are two adrenal glands, one on top of each kidney, in the back of the upper abdomen. The adrenal glands make important hormones that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Neuroblastoma may also begin in the abdomen, chest, spinal cord, or in nerve tissue near the spine in the neck. Neuroblastoma most often begins during early childhood, usually in children younger than 5 years of age. See the PDQ summary on Neuroblastoma Treatment for more information about neuroblastoma. Most cases of neuroblastoma are diagnosed before 1 year of age. Neuroblastoma is the most common type of cancer in infants. The number of new cases of neuroblastoma is greatest among children under 1 year of age. As children get older, the number of new cases decreases. Neuroblastoma is slightly more common in males than females. Neuroblastoma sometimes forms before birth but is usually found later, when the tumor begins to grow and cause symptoms. In rare cases, neuroblastoma may be found before birth, by fetal ultrasound.
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Neuroblastoma
2
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susceptibility
Who is at risk for Neuroblastoma? ?
The risk factors for neuroblastoma are not known.
0000031_2
Neuroblastoma
4
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susceptibility
Who is at risk for Neuroblastoma? ?
Key Points - Screening tests have risks. - The risks of neuroblastoma screening include the following: - Neuroblastoma may be overdiagnosed. - False-negative test results can occur. - False-positive test results can occur. Screening tests have risks. Decisions about screening tests can be difficult. Not all screening tests are helpful and most have risks. Before having any screening test, you may want to discuss the test with your doctor. It is important to know the risks of the test and whether it has been proven to reduce the risk of dying from cancer. The risks of neuroblastoma screening include the following: Neuroblastoma may be overdiagnosed. When a screening test result leads to the diagnosis and treatment of a disease that may never have caused symptoms or become life-threatening, it is called overdiagnosis. For example, when a urine test result shows a higher than normal amount of homovanillic acid (HMA) or vanillyl mandelic acid (VMA), tests and treatments for neuroblastoma are likely to be done, but may not be needed. At this time, it is not possible to know which neuroblastomas found by a screening test will cause symptoms and which neuroblastomas will not. Diagnostic tests (such as biopsies) and cancer treatments (such as surgery, radiation therapy, and chemotherapy) can have serious risks, including physical and emotional problems. False-negative test results can occur. Screening test results may appear to be normal even though neuroblastoma is present. A person who receives a false-negative test result (one that shows there is no cancer when there really is) may delay seeking medical care even if there are symptoms. False-positive test results can occur. Screening test results may appear to be abnormal even though no cancer is present. A false-positive test result (one that shows there is cancer when there really isn't) can cause anxiety and is usually followed by more tests and procedures, which also have risks.
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Langerhans Cell Histiocytosis
1
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information
What is (are) Langerhans Cell Histiocytosis ?
Key Points - Langerhans cell histiocytosis is a type of cancer that can damage tissue or cause lesions to form in one or more places in the body. - Family history or having a parent who was exposed to certain chemicals may increase the risk of LCH. - The signs and symptoms of LCH depend on where it is in the body. - Skin and nails - Mouth - Bone - Lymph nodes and thymus - Endocrine system - Central nervous system (CNS) - Liver and spleen - Lung - Bone marrow - Tests that examine the organs and body systems where LCH may occur are used to detect (find) and diagnose LCH. - Certain factors affect prognosis (chance of recovery) and treatment options. Langerhans cell histiocytosis is a type of cancer that can damage tissue or cause lesions to form in one or more places in the body. Langerhans cell histiocytosis (LCH) is a rare cancer that begins in LCH cells (a type of dendritic cell which fights infection). Sometimes there are mutations (changes) in LCH cells as they form. These include mutations of the BRAF gene. These changes may make the LCH cells grow and multiply quickly. This causes LCH cells to build up in certain parts of the body, where they can damage tissue or form lesions. LCH is not a disease of the Langerhans cells that normally occur in the skin. LCH may occur at any age, but is most common in young children. Treatment of LCH in children is different from treatment of LCH in adults. The treatments for LCH in children and adults are described in separate sections of this summary. Check the list of NCI-supported cancer clinical trials that are now accepting patients with childhood Langerhans cell histiocytosis. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.
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Langerhans Cell Histiocytosis
2
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susceptibility
Who is at risk for Langerhans Cell Histiocytosis? ?
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for LCH include the following: - Having a parent who was exposed to certain chemicals such as benzene. - Having a parent who was exposed to metal, granite, or wood dust in the workplace. - A family history of cancer, including LCH. - Having infections as a newborn. - Having a personal history or family history of thyroid disease. - Smoking, especially in young adults. - Being Hispanic.
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Langerhans Cell Histiocytosis
3
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symptoms
What are the symptoms of Langerhans Cell Histiocytosis ?
These and other signs and symptoms may be caused by LCH or by other conditions. Check with your doctor if you or your child have any of the following: Skin and nails LCH in infants may affect the skin only. In some cases, skin-only LCH may get worse over weeks or months and become a form called high-risk multisystem LCH. In infants, signs or symptoms of LCH that affects the skin may include: - Flaking of the scalp that may look like cradle cap. - Raised, brown or purple skin rash anywhere on the body. In children and adults, signs or symptoms of LCH that affects the skin and nails may include: - Flaking of the scalp that may look like dandruff. - Raised, red or brown, crusted rash in the groin area, abdomen, back, or chest, that may be itchy. - Bumps or ulcers on the scalp. - Ulcers behind the ears, under the breasts, or in the groin area. - Fingernails that fall off or have discolored grooves that run the length of the nail. Mouth Signs or symptoms of LCH that affects the mouth may include: - Swollen gums. - Sores on the roof of the mouth, inside the cheeks, or on the tongue or lips. - Teeth that become uneven. - Tooth loss. Bone Signs or symptoms of LCH that affects the bone may include: - Swelling or a lump over a bone, such as the skull, ribs, spine, thigh bone, upper arm bone, elbow, eye socket, or bones around the ear. - Pain where there is swelling or a lump over a bone. Children with LCH lesions in bones around the ears or eyes have a high risk for diabetes insipidus and other central nervous system disease. Lymph nodes and thymus Signs or symptoms of LCH that affects the lymph nodes or thymus may include: - Swollen lymph nodes. - Trouble breathing. - Superior vena cava syndrome. This can cause coughing, trouble breathing, and swelling of the face, neck, and upper arms. Endocrine system Signs or symptoms of LCH that affects the pituitary gland may include: - Diabetes insipidus. This can cause a strong thirst and frequent urination. - Slow growth. - Early or late puberty. - Being very overweight. Signs or symptoms of LCH that affects the thyroid may include: - Swollen thyroid gland. - Hypothyroidism. This can cause tiredness, lack of energy, being sensitive to cold, constipation, dry skin, thinning hair, memory problems, trouble concentrating, and depression. In infants, this can also cause a loss of appetite and choking on food. In children and adolescents, this can also cause behavior problems, weight gain, slow growth, and late puberty. - Trouble breathing. Central nervous system (CNS) Signs or symptoms of LCH that affects the CNS (brain and spinal cord) may include: - Loss of balance, uncoordinated body movements, and trouble walking. - Trouble speaking. - Trouble seeing. - Headaches. - Changes in behavior or personality. - Memory problems. These signs and symptoms may be caused by lesions in the CNS or by CNS neurodegenerative syndrome. Liver and spleen Signs or symptoms of LCH that affects the liver or spleen may include: - Swelling in the abdomen caused by a buildup of extra fluid. - Trouble breathing. - Yellowing of the skin and whites of the eyes. - Itching. - Easy bruising or bleeding. - Feeling very tired. Lung Signs or symptoms of LCH that affects the lung may include: - Collapsed lung. This condition can cause chest pain or tightness, trouble breathing, feeling tired, and a bluish color to the skin. - Trouble breathing, especially in adults who smoke. - Dry cough. - Chest pain. Bone marrow Signs or symptoms of LCH that affects the bone marrow may include: - Easy bruising or bleeding. - Fever. - Frequent infections.
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Langerhans Cell Histiocytosis
4
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exams and tests
How to diagnose Langerhans Cell Histiocytosis ?
The following tests and procedures may be used to detect (find) and diagnose LCH or conditions caused by LCH: - Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken. - Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person's mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam. - Complete blood count (CBC) with differential : A procedure in which a sample of blood is drawn and checked for the following: - The amount of hemoglobin (the protein that carries oxygen) in the red blood cells. - The portion of the blood sample made up of red blood cells. - The number and type of white blood cells. - The number of red blood cells and platelets. - Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the body by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease. - Liver function test : A blood test to measure the blood levels of certain substances released by the liver. A high or low level of these substances can be a sign of disease in the liver. - BRAF gene testing : A laboratory test in which a sample of blood or tissue is tested for mutations of the BRAF gene. - Urinalysis : A test to check the color of urine and its contents, such as sugar, protein, red blood cells, and white blood cells. - Water deprivation test : A test to check how much urine is made and whether it becomes concentrated when little or no water is given. This test is used to diagnose diabetes insipidus, which may be caused by LCH. - Bone marrow aspiration and biopsy : The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone. A pathologist views the bone marrow and bone under a microscope to look for signs of LCH. The following tests may be done on the tissue that was removed: - Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer. - Flow cytometry : A laboratory test that measures the number of cells in a sample, how many cells are live, and the size of the cells. It also shows the shapes of the cells and whether there are tumor markers on the surface of the cells. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light. - Bone scan : A procedure to check if there are rapidly dividing cells in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner. - X-ray : An x-ray of the organs and bones inside the body. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. Sometimes a skeletal survey is done. This is a procedure to x-ray all of the bones in the body. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. A substance called gadolinium may be injected into a vein. The gadolinium collects around the LCH cells so that they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later. - Bronchoscopy : A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. - Endoscopy : A procedure to look at organs and tissues inside the body to check for abnormal areas in the gastrointestinal tract or lungs. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease. - Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for LCH cells. To diagnose LCH, a biopsy of bone lesions, skin, lymph nodes, or the liver may be done.
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Langerhans Cell Histiocytosis
5
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outlook
What is the outlook for Langerhans Cell Histiocytosis ?
LCH in organs such as the skin, bones, lymph nodes, or pituitary gland usually gets better with treatment and is called "low- risk". LCH in the spleen, liver, or bone marrow is harder to treat and is called "high-risk". The prognosis (chance of recovery) and treatment options depend on the following: - Whether there are mutations of the BRAF gene. - How old the patient is when diagnosed with LCH. - How many organs or body systems the cancer affects. - Whether the cancer is found in the liver, spleen, bone marrow, or certain bones in the skull. - How quickly the cancer responds to initial treatment. - Whether the cancer has just been diagnosed or has come back (recurred). In infants up to one year of age, LCH may go away without treatment.
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Langerhans Cell Histiocytosis
6
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stages
What are the stages of Langerhans Cell Histiocytosis ?
Key Points - There is no staging system for Langerhans cell histiocytosis (LCH). - Treatment of LCH is based on where LCH cells are found in the body and how many body systems are affected. There is no staging system for Langerhans cell histiocytosis (LCH). The extent or spread of cancer is usually described as stages. There is no staging system for LCH. Treatment of LCH is based on where LCH cells are found in the body and how many body systems are affected. LCH is described as single-system disease or multisystem disease, depending on how many body systems are affected: - Single-system LCH: LCH is found in one part of an organ or body system (unifocal) or in more than one part of that organ or body system (multifocal). Bone is the most common single place for LCH to be found. - Multisystem LCH: LCH occurs in two or more organs or body systems or may be spread throughout the body. Multisystem LCH is less common than single-system LCH. LCH may affect low-risk organs or high-risk organs: - Low-risk organs include the skin, bone, lungs, lymph nodes, gastrointestinal tract, pituitary gland, and central nervous system (CNS). - High-risk organs include the liver, spleen, and bone marrow.
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Langerhans Cell Histiocytosis
7
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treatment
What are the treatments for Langerhans Cell Histiocytosis ?
Key Points - There are different types of treatment for patients with Langerhans cell histiocytosis (LCH). - Children with LCH should have their treatment planned by a team of health care providers who are experts in treating childhood cancer. - Some cancer treatments cause side effects months or years after treatment for childhood cancer has ended. - Nine types of standard treatment are used: - Chemotherapy - Surgery - Radiation therapy - Photodynamic therapy - Biologic therapy - Targeted therapy - Other drug therapy - Stem cell transplant - Observation - New types of treatment are being tested in clinical trials. - Patients may want to think about taking part in a clinical trial. - Patients can enter clinical trials before, during, or after starting their treatment. - When treatment of LCH stops, new lesions may appear or old lesions may come back. - Follow-up tests may be needed. There are different types of treatment for patients with Langerhans cell histiocytosis (LCH). Different types of treatments are available for patients with LCH. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Whenever possible, patients should take part in a clinical trial in order to receive new types of treatment for LCH. Some clinical trials are open only to patients who have not started treatment. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI website. Choosing the most appropriate treatment is a decision that ideally involves the patient, family, and health care team. Children with LCH should have their treatment planned by a team of health care providers who are experts in treating childhood cancer. Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric healthcare providers who are experts in treating children with LCH and who specialize in certain areas of medicine. These may include the following specialists: - Pediatrician. - Primary care physician. - Pediatric surgeon. - Pediatric hematologist. - Radiation oncologist. - Neurologist. - Endocrinologist. - Pediatric nurse specialist. - Rehabilitation specialist. - Psychologist. - Social worker. Some cancer treatments cause side effects months or years after treatment for childhood cancer has ended. Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following: - Slow growth and development. - Hearing loss. - Bone, tooth, liver, and lung problems. - Changes in mood, feeling, learning, thinking, or memory. - Second cancers, such as leukemia, retinoblastoma, Ewing sarcoma, brain or liver cancer. Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.) Many patients with multisystem LCH have late effects caused by treatment or by the disease itself. These patients often have long-term health problems that affect their quality of life. Nine types of standard treatment are used: Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly onto the skin or into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Chemotherapy agents given by injection or by mouth are used to treat LCH. Chemotherapy agents include vinblastine, cytarabine, cladribine, and methotrexate. Nitrogen mustard is a drug that is put directly on the skin to treat small LCH lesions. Surgery Surgery may be used to remove LCH lesions and a small amount of nearby healthy tissue. Curettage is a type of surgery that uses a curette (a sharp, spoon-shaped tool) to scrape LCH cells from bone. When there is severe liver or lung damage, the entire organ may be removed and replaced with a healthy liver or lung from a donor. Radiation therapy Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the cancer. In LCH, a special lamp may be used to send ultraviolet B (UVB) radiation toward LCH skin lesions. Photodynamic therapy Photodynamic therapy is a cancer treatment that uses a drug and a certain type of laser light to kill cancer cells. A drug that is not active until it is exposed to light is injected into a vein. The drug collects more in cancer cells than in normal cells. For LCH, laser light is aimed at the skin and the drug becomes active and kills the cancer cells. Photodynamic therapy causes little damage to healthy tissue. Patients who have photodynamic therapy should not spend too much time in the sun. In one type of photodynamic therapy, called psoralen and ultraviolet A (PUVA) therapy, the patient receives a drug called psoralen and then ultraviolet A radiation is directed to the skin. Biologic therapy Biologic therapy is a treatment that uses the patients immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the bodys natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon is a type of biologic therapy used to treat LCH of the skin. Immunomodulators are also a type of biologic therapy. Thalidomide is an immunomodulator used to treat LCH. Targeted therapy Targeted therapy is a type of treatment that uses drugs or other substances to find and attack LCH cells without harming normal cells. Imatinib mesylate is a type of targeted therapy called a tyrosine kinase inhibitor. It stops blood stem cells from turning into dendritic cells that may become cancer cells. Other types of kinase inhibitors that affect cells with mutations (changes) in the BRAF gene, such as dabrafenib and vemurafenib, are being studied in clinical trials for LCH. A family of genes, called ras genes, may cause cancer when they are mutated. Ras genes make proteins that are involved in cell signaling pathways, cell growth, and cell death. Ras pathway inhibitors are a type of targeted therapy being studied in clinical trials. They block the actions of a mutated ras gene or its protein and may stop the growth of cancer. Other drug therapy Other drugs used to treat LCH include the following: - Steroid therapy, such as prednisone, is used to treat LCH lesions. - Bisphosphonate therapy (such as pamidronate, zoledronate, or alendronate) is used to treat LCH lesions of the bone and to lessen bone pain. - Nonsteroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin and ibuprofen) that are commonly used to decrease fever, swelling, pain, and redness. Sometimes an NSAID called indomethacin is used to treat LCH. - Retinoids, such as isotretinoin, are drugs related to vitamin A that can slow the growth of LCH cells in the skin. The retinoids are taken by mouth. Stem cell transplant Stem cell transplant is a method of giving chemotherapy and replacing blood-forming cells destroyed by the LCH treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells. Observation Observation is closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change. New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options for Childhood LCH and the Treatment Options for Adult LCH sections for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database. When treatment of LCH stops, new lesions may appear or old lesions may come back. Many patients with LCH get better with treatment. However, when treatment stops, new lesions may appear or old lesions may come back. This is called reactivation (recurrence) and may occur within one year after stopping treatment. Patients with multisystem disease are more likely to have a reactivation. More common sites of reactivation are bone, ears, or skin. Diabetes insipidus also may develop. Less common sites of reactivation include lymph nodes, bone marrow, spleen, liver, or lung. Some patients may have more than one reactivation over a number of years. Follow-up tests may be needed. Some of the tests that were done to diagnose LCH may be repeated. This is to see how well the treatment is working and if there are any new lesions. These tests may include: - Physical exam. - Neurological exam. - Ultrasound exam. - MRI. - CT scan. - PET scan. Other tests that may be needed include: - Brain stem auditory evoked response (BAER) test: A test that measures the brain's response to clicking sounds or certain tones. - Pulmonary function test (PFT): A test to see how well the lungs are working. It measures how much air the lungs can hold and how quickly air moves into and out of the lungs. It also measures how much oxygen is used and how much carbon dioxide is given off during breathing. This is also called a lung function test. - Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Treatment Options for LCH in Children Treatment of Low-Risk Disease in Children Skin Lesions Treatment of childhood Langerhans cell histiocytosis (LCH) skin lesions may include the following: - Observation. When severe rashes, pain, ulceration, or bleeding occur, treatment may include the following: - Steroid therapy. - Chemotherapy, given by mouth. - Nitrogen mustard applied to the skin. - Photodynamic therapy with psoralen and ultraviolet A (PUVA) therapy. - UVB radiation therapy. Lesions in Bones or Other Low-Risk Organs Treatment of childhood LCH bone lesions in the front, sides, or back of the skull, or in any other single bone may include the following: - Surgery (curettage) with or without steroid therapy. - Low-dose radiation therapy for lesions that affect nearby organs. Treatment of childhood LCH lesions in bones around the ears or eyes is done to lower the risk of diabetes insipidus and other long-term problems. Treatment may include: - Chemotherapy and steroid therapy. - Surgery (curettage). Treatment of childhood LCH lesions of the spine or thigh bone lesions may include: - Observation. - Low-dose radiation therapy. - Chemotherapy, for lesions that spread from the spine into nearby tissue. - Surgery to strengthen the weakened bone by bracing or fusing the bones together. Treatment of two or more bone lesions may include: - Chemotherapy and steroid therapy. Treatment of two or more bone lesions combined with childhood LCH skin lesions, lymph node lesions, or diabetes insipidus may include: - Chemotherapy with or without steroid therapy. - Bisphosphonate therapy. Treatment of High-Risk Disease in Children Treatment of childhood LCH multisystem disease lesions in the spleen, liver, or bone marrow (with or without skin, bone, lymph node, lung, or pituitary gland lesions) may include: - Chemotherapy and steroid therapy. Higher doses of combination chemotherapy and steroid therapy may be given to patients whose tumors do not respond to initial chemotherapy. - A liver transplant for patients with severe liver damage. Treatment of childhood LCH central nervous system (CNS) lesions may include: - Chemotherapy with or without steroid therapy. - Steroid therapy. Treatment of LCH CNS neurodegenerative syndrome may include: - Retinoid therapy. - Chemotherapy. Treatment Options for Recurrent, Refractory, and Progressive Childhood LCH in Children Recurrent LCH is cancer that cannot be detected for some time after treatment and then comes back. Treatment of recurrent childhood LCH in the skin, bone, lymph nodes, gastrointestinal tract, pituitary gland, or central nervous system (low-risk organs) may include: - Chemotherapy with or without steroid therapy. - Bisphosphonate therapy. - Nonsteroidal anti-inflammatory drug (NSAID) therapy with indomethacin. - A clinical trial of a targeted therapy. Refractory LCH is cancer that does not get better with treatment. Treatment of refractory childhood LCH in high-risk organs and in multisystem low-risk organs may include high-dose chemotherapy. Treatment of childhood LCH in multisystem high-risk organs that did not respond to chemotherapy may include stem cell transplant. Progressive LCH is cancer that continues to grow during treatment. Treatment of progressive childhood LCH in patients with multisystem disease may include anticancer drugs that have not been given to the patient before. Treatment Options for LCH in Adults Treatment Options for LCH of the Lung in Adults Treatment for LCH of the lung in adults may include: - Quitting smoking for all patients who smoke. Lung damage will get worse over time in patients who do not quit smoking. In patients who quit smoking, lung damage may get better or it may get worse over time. - Chemotherapy. - Lung transplant for patients with severe lung damage. Sometimes LCH of the lung will go away or not get worse even if it's not treated. Treatment Options for LCH of the Bone in Adults Treatment for LCH that affects only the bone in adults may include: - Surgery with or without steroid therapy. - Chemotherapy with or without low-dose radiation therapy. - Radiation therapy. - Bisphosphonate therapy, for severe bone pain. Treatment Options for LCH of the Skin in Adults Treatment for LCH that affects only the skin in adults may include: - Surgery. - Steroid or other drug therapy applied or injected into the skin. - Photodynamic therapy with psoralen and ultraviolet A (PUVA) radiation. - UVB radiation therapy. - Chemotherapy or biologic therapy given by mouth, such as methotrexate, thalidomide, or interferon. - Retinoid therapy may be used if the skin lesions do not get better with other treatment. Treatment for LCH that affects the skin and other body systems in adults may include: - Chemotherapy. Treatment Options for Single-System and Multisystem LCH in Adults Treatment of single-system and multisystem disease in adults may include: - Chemotherapy with or without a drug given to weaken the immune system. - Bisphosphonate therapy, for severe bone pain. - A clinical trial of a targeted therapy.
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Langerhans Cell Histiocytosis
8
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research
what research (or clinical trials) is being done for Langerhans Cell Histiocytosis ?
New types of treatment are being tested in clinical trials. Information about clinical trials is available from the NCI website. Patients may want to think about taking part in a clinical trial. For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment. Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment. Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward. Patients can enter clinical trials before, during, or after starting their treatment. Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment. Clinical trials are taking place in many parts of the country. See the Treatment Options for Childhood LCH and the Treatment Options for Adult LCH sections for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.
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Liver (Hepatocellular) Cancer
1
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information
What is (are) Liver (Hepatocellular) Cancer ?
Key Points - Liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver. - Liver cancer is not common in the United States. - Being infected with certain types of the hepatitis virus can cause hepatitis and increase the risk of liver cancer. - Hepatitis A - Hepatitis B - Hepatitis C - Hepatitis D - Hepatitis E - Hepatitis G Liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver. The liver is one of the largest organs in the body. It has four lobes and fills the upper right side of the abdomen inside the rib cage. Three of the many important functions of the liver are: - To filter harmful substances from the blood so they can be passed from the body in stools and urine. - To make bile to help digest fats from food. - To store glycogen (sugar), which the body uses for energy. See the following PDQ summaries for more information about liver (hepatocellular) cancer: - Liver (Hepatocellular) Cancer Screening - Adult Primary Liver Cancer Treatment - Childhood Liver Cancer Treatment Liver cancer is not common in the United States. Liver cancer is the fourth most common cancer and the third leading cause of cancer death in the world. In the United States, men, especially Asian/Pacific Islander men, have an increased risk of liver cancer. The number of new cases of liver cancer and the number of deaths from liver cancer continue to increase, especially among middle-aged black, Hispanic, and white men. People are usually older than 40 years when they develop this cancer. Finding and treating liver cancer early may prevent death from liver cancer.
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Liver (Hepatocellular) Cancer
2
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susceptibility
Who is at risk for Liver (Hepatocellular) Cancer? ?
Being infected with certain types of the hepatitis virus can cause hepatitis and increase the risk of liver cancer. Hepatitis is most commonly caused by the hepatitis virus. Hepatitis is a disease that causes inflammation (swelling) of the liver. Damage to the liver from hepatitis that lasts a long time can increase the risk of liver cancer. There are six types of the hepatitis virus. Hepatitis A (HAV), hepatitis B (HBV), and hepatitis C (HCV) are the three most common types. These three viruses cause similar symptoms, but the ways they spread and affect the liver are different. The Hepatitis A vaccine and the hepatitis B vaccine prevent infection with hepatitis A and hepatitis B. There is no vaccine to prevent infection with hepatitis C. If a person has had one type of hepatitis in the past, it is still possible to get the other types. Hepatitis viruses include: Hepatitis A Hepatitis A is caused by eating food or drinking water infected with hepatitis A virus. It does not lead to chronic disease. People with hepatitis A usually get better without treatment. Hepatitis B Hepatitis B is caused by contact with the blood, semen, or other body fluid of a person infected with hepatitis B virus. It is a serious infection that may become chronic and cause scarring of the liver (cirrhosis). This may lead to liver cancer. Blood banks test all donated blood for hepatitis B, which greatly lowers the risk of getting the virus from blood transfusions. Hepatitis C Hepatitis C is caused by contact with the blood of a person infected with hepatitis C virus. Hepatitis C may range from a mild illness that lasts a few weeks to a serious, lifelong illness. Most people who have hepatitis C develop a chronic infection that may cause scarring of the liver (cirrhosis). This may lead to liver cancer. Blood banks test all donated blood for hepatitis C, which greatly lowers the risk of getting the virus from blood transfusions. Hepatitis D Hepatitis D develops in people already infected with hepatitis B. It is caused by hepatitis D virus (HDV) and is spread through contact with infected blood or dirty needles, or by having unprotected sex with a person infected with HDV. Hepatitis D causes acute hepatitis. Hepatitis E Hepatitis E is caused by hepatitis E virus (HEV). Hepatitis E can be spread through oral- anal contact or by drinking infected water. Hepatitis E is rare in the United States. Hepatitis G Being infected with hepatitis G virus (HGV) has not been shown to cause liver cancer. Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following risk factors may increase the risk of liver cancer: - Hepatitis B and C - Cirrhosis - Aflatoxin - The following protective factor may decrease the risk of liver cancer: - Hepatitis B vaccine - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent liver cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. The following risk factors may increase the risk of liver cancer: Hepatitis B and C Having chronic hepatitis B or chronic hepatitis C increases the risk of developing liver cancer. The risk is even greater for people with both hepatitis B and C. Also, the longer the hepatitis infection lasts (especially hepatitis C), the greater the risk. In a study of patients with chronic hepatitis C, those who were treated to lower their iron levels by having blood drawn and eating a low-iron diet were less likely to develop liver cancer than those who did not have this treatment. Cirrhosis The risk of developing liver cancer is increased for people who have cirrhosis, a disease in which healthy liver tissue is replaced by scar tissue. The scar tissue blocks the flow of blood through the liver and keeps it from working as it should. Chronic alcoholism and chronic hepatitis C are the most common causes of cirrhosis. Aflatoxin The risk of developing liver cancer may be increased by eating foods that contain aflatoxin (poison from a fungus that can grow on foods, such as grains and nuts, that have not been stored properly).
0000007_4
Liver (Hepatocellular) Cancer
3
0000007_4-3
prevention
How to prevent Liver (Hepatocellular) Cancer ?
Key Points - Avoiding risk factors and increasing protective factors may help prevent cancer. - The following risk factors may increase the risk of liver cancer: - Hepatitis B and C - Cirrhosis - Aflatoxin - The following protective factor may decrease the risk of liver cancer: - Hepatitis B vaccine - Cancer prevention clinical trials are used to study ways to prevent cancer. - New ways to prevent liver cancer are being studied in clinical trials. Avoiding risk factors and increasing protective factors may help prevent cancer. Avoiding cancer risk factors may help prevent certain cancers. Risk factors include smoking, being overweight, and not getting enough exercise. Increasing protective factors such as quitting smoking and exercising may also help prevent some cancers. Talk to your doctor or other health care professional about how you might lower your risk of cancer. The following risk factors may increase the risk of liver cancer: Hepatitis B and C Having chronic hepatitis B or chronic hepatitis C increases the risk of developing liver cancer. The risk is even greater for people with both hepatitis B and C. Also, the longer the hepatitis infection lasts (especially hepatitis C), the greater the risk. In a study of patients with chronic hepatitis C, those who were treated to lower their iron levels by having blood drawn and eating a low-iron diet were less likely to develop liver cancer than those who did not have this treatment. Cirrhosis The risk of developing liver cancer is increased for people who have cirrhosis, a disease in which healthy liver tissue is replaced by scar tissue. The scar tissue blocks the flow of blood through the liver and keeps it from working as it should. Chronic alcoholism and chronic hepatitis C are the most common causes of cirrhosis. Aflatoxin The risk of developing liver cancer may be increased by eating foods that contain aflatoxin (poison from a fungus that can grow on foods, such as grains and nuts, that have not been stored properly). The following protective factor may decrease the risk of liver cancer: Hepatitis B vaccine Preventing hepatitis B infection (by being vaccinated for hepatitis B) has been shown to lower the risk of liver cancer in children. It is not yet known if it lowers the risk in adults. Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent liver cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for liver cancer prevention trials that are now accepting patients.
0000007_4
Liver (Hepatocellular) Cancer
4
0000007_4-4
research
what research (or clinical trials) is being done for Liver (Hepatocellular) Cancer ?
Cancer prevention clinical trials are used to study ways to prevent cancer. Cancer prevention clinical trials are used to study ways to lower the risk of developing certain types of cancer. Some cancer prevention trials are conducted with healthy people who have not had cancer but who have an increased risk for cancer. Other prevention trials are conducted with people who have had cancer and are trying to prevent another cancer of the same type or to lower their chance of developing a new type of cancer. Other trials are done with healthy volunteers who are not known to have any risk factors for cancer. The purpose of some cancer prevention clinical trials is to find out whether actions people take can prevent cancer. These may include eating fruits and vegetables, exercising, quitting smoking, or taking certain medicines, vitamins, minerals, or food supplements. New ways to prevent liver cancer are being studied in clinical trials. Clinical trials are taking place in many parts of the country. Information about clinical trials can be found in the Clinical Trials section of the NCI website. Check NCI's list of cancer clinical trials for liver cancer prevention trials that are now accepting patients.
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Chronic Neutrophilic Leukemia
1
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information
What is (are) Chronic Neutrophilic Leukemia ?
Chronic neutrophilic leukemia is a disease in which too many blood stem cells become a type of white blood cell called neutrophils. Neutrophils are infection -fighting blood cells that surround and destroy dead cells and foreign substances (such as bacteria). The spleen and liver may swell because of the extra neutrophils. Chronic neutrophilic leukemia may stay the same or it may progress quickly to acute leukemia.
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Chronic Neutrophilic Leukemia
2
0000013_2-2
treatment
What are the treatments for Chronic Neutrophilic Leukemia ?
Treatment of chronic neutrophilic leukemia may include the following: - Donor bone marrow transplant. - Chemotherapy. - Biologic therapy using interferon alfa. - A clinical trial of a new treatment. Check the list of NCI-supported cancer clinical trials that are now accepting patients with chronic neutrophilic leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI website.
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Vulvar Cancer
1
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information
What is (are) Vulvar Cancer ?
Key Points - Vulvar cancer is a rare disease in which malignant (cancer) cells form in the tissues of the vulva. - Having vulvar intraepithelial neoplasia or HPV infection can affect the risk of vulvar cancer. - Signs of vulvar cancer include bleeding or itching. - Tests that examine the vulva are used to detect (find) and diagnose vulvar cancer. - Certain factors affect prognosis (chance of recovery) and treatment options. Vulvar cancer is a rare disease in which malignant (cancer) cells form in the tissues of the vulva. Vulvar cancer forms in a woman's external genitalia. The vulva includes: - Inner and outer lips of the vagina. - Clitoris (sensitive tissue between the lips). - Opening of the vagina and its glands. - Mons pubis (the rounded area in front of the pubic bones that becomes covered with hair at puberty). - Perineum (the area between the vulva and the anus). Vulvar cancer most often affects the outer vaginal lips. Less often, cancer affects the inner vaginal lips, clitoris, or vaginal glands. Vulvar cancer usually forms slowly over a number of years. Abnormal cells can grow on the surface of the vulvar skin for a long time. This condition is called vulvar intraepithelial neoplasia (VIN). Because it is possible for VIN to become vulvar cancer, it is very important to get treatment. Having vulvar intraepithelial neoplasia or HPV infection can affect the risk of vulvar cancer. Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for vulvar cancer include the following: - Having vulvar intraepithelial neoplasia (VIN). - Having human papillomavirus (HPV) infection. - Having a history of genital warts. Other possible risk factors include the following: - Having many sexual partners. - Having first sexual intercourse at a young age. - Having a history of abnormal Pap tests (Pap smears). Recurrent Vulvar Cancer Recurrent vulvar cancer is cancer that has recurred (come back) after it has been treated. The cancer may come back in the vulva or in other parts of the body.
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Vulvar Cancer
2
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symptoms
What are the symptoms of Vulvar Cancer ?
Signs of vulvar cancer include bleeding or itching. Vulvar cancer often does not cause early signs or symptoms. Signs and symptoms may be caused by vulvar cancer or by other conditions. Check with your doctor if you have any of the following: - A lump or growth on the vulva. - Changes in the vulvar skin, such as color changes or growths that look like a wart or ulcer. - Itching in the vulvar area, that does not go away. - Bleeding not related to menstruation (periods). - Tenderness in the vulvar area.
0000043_1
Vulvar Cancer
3
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exams and tests
How to diagnose Vulvar Cancer ?
Tests that examine the vulva are used to detect (find) and diagnose vulvar cancer. The following tests and procedures may be used: - Physical exam and history : An exam of the body to check general signs of health, including checking the vulva for signs of disease, such as lumps or anything else that seems unusual. A history of the patients health habits and past illnesses and treatments will also be taken. - Biopsy : The removal of samples of cells or tissues from the vulva so they can be viewed under a microscope by a pathologist to check for signs of cancer.
0000043_1
Vulvar Cancer
4
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outlook
What is the outlook for Vulvar Cancer ?
Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on the following: - The stage of the cancer. - The patient's age and general health. - Whether the cancer has just been diagnosed or has recurred (come back).
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Vulvar Cancer
5
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stages
What are the stages of Vulvar Cancer ?
Key Points - After vulvar cancer has been diagnosed, tests are done to find out if cancer cells have spread within the vulva or to other parts of the body. - There are three ways that cancer spreads in the body. - Cancer may spread from where it began to other parts of the body. - In vulvar intraepithelial neoplasia (VIN), abnormal cells are found on the surface of the vulvar skin. - The following stages are used for vulvar cancer: - Stage I - Stage II - Stage III - Stage IV After vulvar cancer has been diagnosed, tests are done to find out if cancer cells have spread within the vulva or to other parts of the body. The process used to find out if cancer has spread within the vulva or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process: - Pelvic exam : An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. A speculum is inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test of the cervix is usually done. The doctor or nurse also inserts one or two lubricated, gloved fingers of one hand into the vagina and places the other hand over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas. - Colposcopy : A procedure in which a colposcope (a lighted, magnifying instrument) is used to check the vagina and cervix for abnormal areas. Tissue samples may be taken using a curette (spoon-shaped instrument) or a brush and checked under a microscope for signs of disease. - Cystoscopy : A procedure to look inside the bladder and urethra to check for abnormal areas. A cystoscope is inserted through the urethra into the bladder. A cystoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. - Proctoscopy : A procedure to look inside the rectum and anus to check for abnormal areas. A proctoscope is inserted into the anus and rectum. A proctoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. - X-rays : An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. To stage vulvar cancer, x-rays may be taken of the organs and bones inside the chest, and the pelvic bones. - Intravenous pyelogram (IVP): A series of x-rays of the kidneys, ureters, and bladder to find out if cancer has spread to these organs. A contrast dye is injected into a vein. As the contrast dye moves through the kidneys, ureters and bladder, x-rays are taken to see if there are any blockages. This procedure is also called intravenous urography. - CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. - MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). - PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. - Sentinel lymph node biopsy : The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. Sentinel lymph node biopsy may be done during surgery to remove the tumor for early-stage vulvar cancer. There are three ways that cancer spreads in the body. Cancer can spread through tissue, the lymph system, and the blood: - Tissue. The cancer spreads from where it began by growing into nearby areas. - Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body. - Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body. Cancer may spread from where it began to other parts of the body. When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood. - Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body. - Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body. The metastatic tumor is the same type of cancer as the primary tumor. For example, if vulvar cancer spreads to the lung, the cancer cells in the lung are actually vulvar cancer cells. The disease is metastatic vulvar cancer, not lung cancer. In vulvar intraepithelial neoplasia (VIN), abnormal cells are found on the surface of the vulvar skin. These abnormal cells are not cancer. Vulvar intraepithelial neoplasia (VIN) may become cancer and spread into nearby tissue. VIN is sometimes called stage 0 or carcinoma in situ. The following stages are used for vulvar cancer: Stage I In stage I, cancer has formed. The tumor is found only in the vulva or perineum (area between the rectum and the vagina). Stage I is divided into stages IA and IB. - In stage IA, the tumor is 2 centimeters or smaller and has spread 1 millimeter or less into the tissue of the vulva. Cancer has not spread to the lymph nodes. - In stage IB, the tumor is larger than 2 centimeters or has spread more than 1 millimeter into the tissue of the vulva. Cancer has not spread to the lymph nodes. Stage II In stage II, the tumor is any size and has spread into the lower part of the urethra, the lower part of the vagina, or the anus. Cancer has not spread to the lymph nodes. Stage III In stage III, the tumor is any size and may have spread into the lower part of the urethra, the lower part of the vagina, or the anus. Cancer has spread to one or more nearby lymph nodes. Stage III is divided into stages IIIA, IIIB, and IIIC. - In stage IIIA, cancer is found in 1 or 2 lymph nodes that are smaller than 5 millimeters or in one lymph node that is 5 millimeters or larger. - In stage IIIB, cancer is found in 2 or more lymph nodes that are 5 millimeters or larger, or in 3 or more lymph nodes that are smaller than 5 millimeters. - In stage IIIC, cancer is found in lymph nodes and has spread to the outside surface of the lymph nodes. Stage IV In stage IV, the tumor has spread into the upper part of the urethra, the upper part of the vagina, or to other parts of the body. Stage IV is divided into stages IVA and IVB. - In stage IVA: - cancer has spread into the lining of the upper urethra, the upper vagina, the bladder, or the rectum, or has attached to the pelvic bone; or - cancer has spread to nearby lymph nodes and the lymph nodes are not moveable or have formed an ulcer. - In stage IVB, cancer has spread to lymph nodes in the pelvis or to other parts of the body.