Adrenocortical Carcinoma
General Information About Adrenocortical Carcinoma
Adrenocortical carcinoma is a rare disease in which malignant (cancer) cells form in the outer layer of the adrenal gland.
There are two adrenal glands. The adrenal glands are small and shaped like a triangle. One adrenal gland sits on top of each kidney. Each adrenal gland has two parts. The outer layer of the adrenal gland is the adrenal cortex. The center of the adrenal gland is the adrenal medulla.
The adrenal cortex makes important hormones that:
- Balance the water and salt in the body.
- Help keep blood pressure normal.
- Help manage the body's use of protein, fat, and carbohydrates.
- Cause the body to have masculine or feminine characteristics.
Adrenocortical carcinoma is also called cancer of the adrenal cortex. A tumor of the adrenal cortex may be functioning (makes more hormones than normal) or nonfunctioning (does not make hormones). The hormones made by functioning tumors may cause certain signs or symptoms of disease.
Cancer that forms in the adrenal medulla is called pheochromocytoma.
Having certain genetic conditions increases the risk of developing adrenocortical 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. People who think they may be at risk should discuss this with their doctor. Risk factors for adrenocortical carcinoma include having the following hereditary diseases:
Possible signs of adrenocortical carcinoma include pain in the abdomen and certain physical changes.
These and other symptoms may be caused by adrenocortical carcinoma:
- A lump in the abdomen.
- Pain the abdomen or back.
A functioning adrenocortical tumor makes too much of a certain hormone (cortisol, aldosterone, testosterone, or estrogen).
Too much cortisol may cause:
- Weight gain in the face, neck, and trunk of the body and thin arms and legs.
- Growth of fine hair on the face, upper back, or arms.
- A round, red, full face.
- A lump of fat on the back of the neck.
- Muscle weakness.
- High blood sugar.
- High blood pressure.
- Muscle weakness or cramps.
- Frequent urination.
- Feeling thirsty.
- Growth of fine hair on the face, upper back, or arms.
- Acne.
- Balding.
- A deepening of the voice.
Too much estrogen (in women) may cause:
- Irregular menstrual periods in women who have not gone through menopause.
- Menstrual bleeding in women who have gone through menopause.
- Growth of breast tissue.
- Lower sex drive.
Imaging studies and tests that examine the blood and urine are used to detect (find) and diagnose adrenocortical carcinoma.
The tests and procedures used to diagnose adrenocortical carcinoma depend on the patient's symptoms. 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 health habits and past illnesses and treatments will also be taken.
- Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of cortisol or 17-ketosteroids. A higher than normal amount of these in the urine may be a sign of disease in the adrenal cortex.
- Low-dose dexamethasone suppression test: A test in which one or more small doses of dexamethasone is given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days.
- High-dose dexamethasone suppression test: A test in which one or more high doses of dexamethasone is given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days.
- Blood chemistry study: A procedure in which a blood sample is checked to measure the amounts of certain substances, such as potassium or sodium, 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.
- Blood tests: Tests to measure the levels of testosterone or estrogen in the blood. A higher than normal amount of these hormones that may be a sign of adrenocortical carcinoma.
- 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). An MRI of the abdomen is done to diagnose adrenocortical carcinoma.
- Adrenal angiography: A procedure to look at the arteries and the flow of blood near the adrenal gland. A contrast dye is injected into the adrenal arteries. As the dye moves through the blood vessel, a series of x-rays are taken to see if any arteries are blocked.
- Adrenal venography: A procedure to look at the adrenal veins and the flow of blood near the adrenal gland. A contrast dye is injected into an adrenal vein. As the contrast dye moves through the vein, a series of x-rays are taken to see if any veins are blocked. A catheter (very thin tube) may be inserted into the vein to take a blood sample, which is checked for abnormal hormone levels.
- 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.
Certain factors affect the 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 is in the adrenal gland only or has spread to other places in the body).
- Whether the tumor can be completely removed in surgery.
- Whether the cancer has been treated in the past.
- The patient's general health.
Stages of Adrenocortical Carcinoma
After adrenocortical carcinoma has been diagnosed, tests are done to find out if cancer cells have spread within the adrenal gland or to other parts of the body.
The process used to find out if cancer has spread within the adrenal gland 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, such as the abdomen or chest, 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) 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. A substance called gadolinium may be 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).
- Adrenal angiography: A procedure to look at the arteries and the flow of blood near the adrenal gland. A contrast dye is injected into the adrenal arteries. As the dye moves through the blood vessel, a series of x-rays are taken to see if any arteries are blocked.
- Adrenal venography: A procedure to look at the adrenal veins and the flow of blood near the adrenal gland. A contrast dye is injected into an adrenal vein. As the contrast dye moves through the vein, x-rays are taken to see if any veins are blocked. A catheter (very thin tube) may be inserted into the vein to take a blood sample, which is checked for abnormal hormone levels.
- Cavagram: A procedure to look at the inferior vena cava and the flow of blood through the inferior vena cava. A contrast dye is injected into a blood vessel. As the contrast dye moves through the blood vessel to the inferior vena cava, a series of x-rays are taken to see if there are any changes to the inferior vena cava and the flow of blood through the inferior vena cava.
- Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs, such as the vena cava, and make echoes. The echoes form a picture of body tissues called a sonogram.
- Adrenalectomy: A procedure to remove the entire adrenal gland. A tissue sample is viewed under a microscope by a pathologist to check for signs of cancer.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
- Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
- Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.
The following stages are used for adrenocortical carcinoma:
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Stage I
In stage I, the tumor is 5 centimeters or smaller and is found only in the adrenal gland.
Stage II
In stage II, the tumor is larger than 5 centimeters and is found only in the adrenal gland.
Stage III
In stage III, the tumor can be any size and may have spread to fat or lymph nodes near the adrenal gland.
Stage IV
In stage IV, the tumor can be any size and has spread:
- to fat or organs and to lymph nodes near the adrenal gland; or
- to other parts of the body. Adrenocortical carcinoma commonly spreads to the lung, liver, bones, and peritoneum (the tissue that lines the abdominal wall and covers most of the organs in the abdomen).
Recurrent Adrenocortical Carcinoma
Recurrent adrenocortical carcinoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the adrenal cortex or in other parts of the body.Treatment Option Overview
There are different types of treatment for patients with adrenocortical carcinoma.
Different types of treatments are available for patients with adrenocortical 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
Surgery to remove the adrenal gland (adrenalectomy) is often used to treat adrenocortical carcinoma. Sometimes the nearby lymph nodes are also removed.
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.
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 spinal column, 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.
Mitotane may be used to treat adrenocortical carcinoma. Mitotane stops the adrenal cortex from making hormones and relieves symptoms caused by the hormones.
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 Web site.
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.
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. 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 cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options by Stage
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.Stage I Adrenocortical Carcinoma
Treatment of stage I adrenocortical carcinoma is usually surgery (adrenalectomy). Lymph nodes may be removed if they are larger than normal.Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage I adrenocortical 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. General information about clinical trials is available from the NCI Web site.
Stage II Adrenocortical Carcinoma
Treatment of stage II adrenocortical carcinoma is usually surgery (adrenalectomy). Lymph nodes may be removed if they are larger than normal.Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage II adrenocortical 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. General information about clinical trials is available from the NCI Web site.
Stage III Adrenocortical Carcinoma
Treatment of stage III adrenocortical carcinoma may include the following:- Surgery (adrenalectomy with or without removal of lymph nodes).
- A clinical trial of radiation therapy for tumors that cannot be removed by surgery.
- A clinical trial of mitotane for tumors that cannot be completely removed by surgery.
Stage IV Adrenocortical Carcinoma
Treatment of stage IV adrenocortical carcinoma may include the following as palliative therapy to relieve symptoms and improve the quality of life:- Mitotane therapy.
- Radiation therapy to bones where cancer has spread.
- Chemotherapy followed by surgery to remove the tumor.
- Surgery to remove the tumor.
- Surgery to remove cancer from places where it has spread.
- A clinical trial of chemotherapy or biologic therapy.
Treatment Options for Recurrent Adrenocortical Carcinoma
Treatment of recurrent adrenocortical carcinoma may include the following as palliative therapy to relieve symptoms and improve the quality of life:- Surgery to remove the cancer from places where it has spread.
- A clinical trial of chemotherapy or biologic therapy.
To Learn More About Adrenocortical Carcinoma
For more information from the National Cancer Institute about adrenocortical carcinoma, see the Adrenocortical Carcinoma Home Page.For general cancer information and other resources from the National Cancer Institute, see the following:
Anal Cancer
General Information About Anal Cancer
Anal cancer is a disease in which malignant (cancer) cells form in the tissues of the anus.
The anus is the end of the large intestine, below the rectum, through which stool (solid waste) leaves the body. The anus is formed partly from the outer, skin layers of the body and partly from the intestine. Two ring-like muscles, called sphincter muscles, open and close the anal opening to let stool pass out of the body. The anal canal, the part of the anus between the rectum and the anal opening, is about 1½ inches long.
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Being infected with the human papillomavirus (HPV) can affect the risk of developing anal cancer.
Risk factors include the following:
- Being over 50 years old.
- Being infected with human papillomavirus (HPV).
- Having many sexual partners.
- Having receptive anal intercourse (anal sex).
- Frequent anal redness, swelling, and soreness.
- Having anal fistulas (abnormal openings).
- Smoking cigarettes.
Possible signs of anal cancer include bleeding from the anus or rectum or a lump near the anus.
These and other symptoms may be caused by anal cancer. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:
- Bleeding from the anus or rectum.
- Pain or pressure in the area around the anus.
- Itching or discharge from the anus.
- A lump near the anus.
- A change in bowel habits.
Tests that examine the rectum and anus are used to detect (find) and diagnose anal 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 lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
- Digital rectal examination (DRE): An exam of the anus and rectum. The doctor or nurse inserts a lubricated, gloved finger into the lower part of the rectum to feel for lumps or anything else that seems unusual.
- Anoscopy: An exam of the anus and lower rectum using a short, lighted tube called an anoscope.
- Proctoscopy: An exam of the rectum using a short, lighted tube called a proctoscope.
- Endo-anal or endorectal ultrasound: A procedure in which an ultrasound transducer (probe) is inserted into the anus or rectum 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.
- Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. If an abnormal area is seen during the anoscopy, a biopsy may be done at that time.
Certain factors affect the prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) depends on the following:
- The size of the tumor.
- Where the tumor is in the anus.
- Whether the cancer has spread to the lymph nodes.
- The stage of the cancer.
- Where the tumor is in the anus.
- Whether the patient has human immunodeficiency virus (HIV).
- Whether cancer remains after initial treatment or has recurred.
Stages of Anal Cancer
After anal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the anus or to other parts of the body.
The process used to find out if cancer has spread within the anus 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 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 anal cancer, a CT scan of the pelvis and abdomen may be done.
- 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.
- Endo-anal or endorectal ultrasound: A procedure in which an ultrasound transducer (probe) is inserted into the anus or rectum 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.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
- Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
- Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.
The following stages are used for anal cancer:
Stage 0 (Carcinoma in Situ)
In stage 0, abnormal cells are found in the innermost lining of the anus. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ.
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Stage I
In stage I, cancer has formed and the tumor is 2 centimeters or smaller.
Stage II
In stage II, the tumor is larger than 2 centimeters.
Stage IIIA
In stage IIIA, the tumor may be any size and has spread to either:
- lymph nodes near the rectum; or
Stage IIIB
In stage IIIB, the tumor may be any size and has spread:
- to nearby organs and to lymph nodes near the rectum; or
- to lymph nodes near the rectum and in the groin, and/or to lymph nodes on both sides of the pelvis and/or groin, and may have spread to nearby organs.
Stage IV
In stage IV, the tumor may be any size and cancer may have spread to lymph nodes or nearby organs and has spread to distant parts of the body.
Recurrent Anal Cancer
Recurrent anal cancer is cancer that has recurred (come back) after it has been treated. The cancer may come back in the anus or in other parts of the body.Treatment Option Overview
There are different types of treatment for patients with anal cancer.
Different types of treatments are available for patients with anal 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. 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.
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 spinal column, 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.
Surgery
- Local resection: A surgical procedure in which the tumor is cut from the anus along with some of the healthy tissue around it. Local resection may be used if the cancer is small and has not spread. This procedure may save the sphincter muscles so the patient can still control bowel movements. Tumors that develop in the lower part of the anus can often be removed with local resection.
- Abdominoperineal resection: A surgical procedure in which the anus, the rectum, and part of the sigmoid colon are removed through an incision made in the abdomen. The doctor sews the end of the intestine to an opening, called a stoma, made in the surface of the abdomen so body waste can be collected in a disposable bag outside of the body. This is called a colostomy. Lymph nodes that contain cancer may also be removed during this operation.
Anal cancer surgery with colostomy. The anus, rectum, and part of the colon are removed, a stoma is created, and a colostomy bag is attached to the stoma. | View a Larger Version 
Having the human immunodeficiency virus can affect treatment of anal cancer.
Cancer therapy can further damage the already weakened immune systems of patients who have the human immunodeficiency virus (HIV). For this reason, patients who have anal cancer and HIV are usually treated with lower doses of anticancer drugs and radiation than patients who do not have HIV.
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 Web site.
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. 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 cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options by Stage
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.Stage 0 (Carcinoma in Situ)
Treatment of stage 0 is usually local resection.Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage 0 anal 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. General information about clinical trials is available from the NCI Web site.
Stage I Anal Cancer
Treatment of stage I anal cancer may include the following:- External-beam radiation therapy with or without chemotherapy. If cancer remains after treatment, additional chemotherapy and radiation therapy may be given to avoid the need for a permanent colostomy.
- Abdominoperineal resection, if cancer remains or comes back after treatment with radiation therapy and chemotherapy.
- Internal radiation therapy for cancer that remains after treatment with external-beam radiation therapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage I anal 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. General information about clinical trials is available from the NCI Web site.
Stage II Anal Cancer
Treatment of stage II anal cancer may include the following:- External-beam radiation therapy with chemotherapy. If cancer remains after treatment, additional chemotherapy and radiation therapy may be given to avoid the need for a permanent colostomy.
- Abdominoperineal resection, if cancer remains or comes back after treatment with radiation therapy and chemotherapy.
- A clinical trial of new treatment options.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage II anal 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. General information about clinical trials is available from the NCI Web site.
Stage IIIA Anal Cancer
Treatment of stage IIIA anal cancer may include the following:- External-beam radiation therapy with chemotherapy. If cancer remains after treatment, additional chemotherapy and radiation therapy may be given to avoid the need for a permanent colostomy.
- Abdominoperineal resection, if cancer remains or comes back after treatment with chemotherapy and radiation therapy.
- A clinical trial of new treatment options.
Stage IIIB Anal Cancer
Treatment of stage IIIB anal cancer may include the following:- Local resection or abdominoperineal resection, if cancer remains or comes back after treatment with chemotherapy and radiation therapy. Lymph nodes may also be removed.
- A clinical trial of new treatment options.
Stage IV Anal Cancer
Treatment of stage IV anal cancer may include the following:- Surgery as palliative therapy to relieve symptoms and improve the quality of life.
- Radiation therapy as palliative therapy.
- Chemotherapy with radiation therapy as palliative therapy.
- A clinical trial of new treatment options.
Treatment Options for Recurrent Anal Cancer
Treatment of recurrent anal cancer may include the following:- Radiation therapy and chemotherapy, for recurrence after surgery.
- Surgery, for recurrence after radiation therapy and/or chemotherapy.
- A clinical trial of radiation therapy with chemotherapy and/or radiosensitizers.
To Learn More About Anal Cancer
For more information from the National Cancer Institute about anal cancer, see the following:- Smoking and Cancer Home Page (Includes help with quitting)
Bladder Cancer
General Information About Bladder Cancer
Bladder cancer is a disease in which malignant (cancer) cells form in the tissues of the bladder.
The bladder is a hollow organ in the lower part of the abdomen. It is shaped like a small balloon and has a muscular wall that allows it to get larger or smaller. The bladder stores urine until it is passed out of the body. Urine is the liquid waste that is made by the kidneys when they clean the blood. The urine passes from the two kidneys into the bladder through two tubes called ureters. When the bladder is emptied during urination, the urine goes from the bladder to the outside of the body through another tube called the urethra.
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- Transitional cell carcinoma: Cancer that begins in cells in the innermost tissue layer of the bladder. These cells are able to stretch when the bladder is full and shrink when it is emptied. Most bladder cancers begin in the transitional cells.
- Squamous cell carcinoma: Cancer that begins in squamous cells, which are thin, flat cells that may form in the bladder after long-term infection or irritation.
- Adenocarcinoma: Cancer that begins in glandular (secretory) cells that may form in the bladder after long-term irritation and inflammation.
See the following PDQ summaries for more information:
Smoking, gender, and diet can affect the risk of developing bladder cancer.
Anything that increases your chance of getting a disease is called a risk factor. Risk factors for bladder cancer include the following:
- Smoking.
- Being exposed to certain substances at work, such as rubber, certain dyes and textiles, paint, and hairdressing supplies.
- A diet high in fried meats and fat.
- Being older, male, or white.
- Having an infection caused by a certain parasite.
Possible signs of bladder cancer include blood in the urine or pain during urination.
These and other symptoms may be caused by bladder cancer. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:
- Blood in the urine (slightly rusty to bright red in color).
- Frequent urination, or feeling the need to urinate without being able to do so.
- Pain during urination.
- Lower back pain.
Tests that examine the urine, vagina, or rectum are used to help detect (find) and diagnose bladder cancer.
The following tests and procedures may be used:
- 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.
- Urinalysis: A test to check the color of urine and its contents, such as sugar, protein, red blood cells, and white blood cells.
- Internal exam: An exam of the vagina and/or rectum. The doctor inserts gloved fingers into the vagina and/or rectum to feel for lumps.
- Intravenous pyelogram (IVP): A series of x-rays of the kidneys, ureters, and bladder to find out if cancer is present in 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.
- 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.
Cystoscopy. A cystoscope (a thin, tube-like instrument with a light and a lens for viewing) is inserted through the urethra into the bladder. Fluid is used to fill the bladder. The doctor looks at an image of the inner wall of the bladder on a computer monitor. | View a Larger Version 
- Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. A biopsy for bladder cancer is usually done during cystoscopy. It may be possible to remove the entire tumor during biopsy.
- Urine cytology: Examination of urine under a microscope to check for abnormal cells.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) depends on the following:
- The stage of the cancer (whether it is superficial or invasive bladder cancer, and whether it has spread to other places in the body). Bladder cancer in the early stages can often be cured.
- The type of bladder cancer cells and how they look under a microscope.
- The patient’s age and general health.
Stages of Bladder Cancer
After bladder cancer has been diagnosed, tests are done to find out if cancer cells have spread within the bladder or to other parts of the body.
The process used to find out if cancer has spread within the bladder lining and muscle 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:
- 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.
Cystoscopy. A cystoscope (a thin, tube-like instrument with a light and a lens for viewing) is inserted through the urethra into the bladder. Fluid is used to fill the bladder. The doctor looks at an image of the inner wall of the bladder on a computer monitor. | View a Larger Version
- 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).
- 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.
- 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.
- Bone scan: A procedure to check if there are rapidly dividing
Adult Brain Tumor
General Information About Adult Brain Tumors
An adult brain tumor is a disease in which abnormal cells form in the tissues of the brain.
There are many types of brain and spinal cord tumors. The tumors are formed by the abnormal growth of cells and may begin in different parts of the brain or spinal cord. Together, the brain and spinal cord make up the central nervous system (CNS).
The tumors may be benign (not cancer) or malignant (cancer). Benign brain tumors grow and press on nearby areas of the brain. They rarely spread into other tissues and may recur (come back). Malignant brain tumors are likely to grow quickly and spread into other brain tissue. When a tumor grows into or presses on an area of the brain, it may stop that part of the brain from working the way it should. Both benign and malignant brain tumors can cause symptoms and need treatment.
Brain tumors can occur in both adults and children. However, treatment for children may be different than treatment for adults. (See the PDQ summary on Childhood Brain and Spinal Cord Tumors Treatment Overview for more information on the treatment of children.)
For information about lymphoma that begins in the brain, see the PDQ summary on Primary CNS Lymphoma Treatment.
A brain tumor that starts in another part of the body and spreads to the brain is called a metastatic tumor.
Tumors that start in the brain are called primary brain tumors. Often, tumors found in the brain have started somewhere else in the body and spread to one or more parts of the brain. These are called metastatic brain tumors (or brain metastases). Metastatic brain tumors are more common than primary brain tumors.
The types of cancer that commonly spread to the brain are melanoma and cancer of the breast, colon, lung, and unknown primary site. The types of cancer that commonly spread to the spinal cord are lymphoma and cancer of the lung, breast, and prostate. About half of metastatic brain and spinal cord tumors are caused by lung cancer. Leukemia, lymphoma, breast cancer, and gastrointestinal cancer may spread to the leptomeninges (the two innermost membranes covering the brain and spinal cord).
See the following PDQ summaries for more information on cancers that commonly spread to the brain and spinal cord:
The brain controls many important body functions.
The brain has three major parts:
- The cerebrum is the largest part of the brain. It is at the top of the head. The cerebrum controls thinking, learning, problem solving, emotions, speech, reading, writing, and voluntary movement.
- The cerebellum is in the lower back of the brain (near the middle of the back of the head). It controls movement, balance, and posture.
- The brain stem connects the brain to the spinal cord. It is in the lowest part of the brain (just above the back of the neck). The brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating.
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The spinal cord connects the brain to nerves in most parts of the body.
The spinal cord is a column of nerve tissue that runs from the brain stem down the center of the back. It is covered by three thin layers of tissue called membranes. These membranes are surrounded by the vertebrae (back bones). Spinal cord nerves carry messages between the brain and the rest of the body, such as a signal from the brain to cause muscles to move or from the skin to the brain about the sense of touch.
There are different types of brain and spinal cord tumors.
Brain and spinal cord tumors are named based on the type of cell they formed in and where the tumor first formed in the CNS. The grade of a tumor may be used to tell the difference between slow- and fast-growing types of the tumor. The grade of a tumor is based on how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread.
- Grade I (low-grade) — The tumor grows slowly, has cells that look a lot like normal cells, and rarely spreads into nearby tissues. It may be possible to remove the entire tumor by surgery.
- Grade II — The tumor grows slowly, but may spread into nearby tissue and may recur (come back). Some tumors may become a higher-grade tumor.
- Grade III — The tumor grows quickly, is likely to spread into nearby tissue, and the tumor cells look very different from normal cells.
- Grade IV (high-grade) — The tumor grows and spreads very quickly and the cells do not look like normal cells. There may be areas of dead cells in the tumor. Grade IV brain tumors are harder to cure than lower-grade tumors.
Astrocytic Tumors
An astrocytic tumor begins in star-shaped brain cells called astrocytes, which help keep nerve cells healthy. An astrocyte is a type of glial cell and is sometimes called a glioma. Astrocytic tumors include the following:
- Brain stem glioma: A brain stem glioma forms in the brain stem, which is the part of the brain connected to the spinal cord. It is often a high-grade tumor, which spreads widely through the brain stem and is hard to cure. A brain stem glioma rarely occurs in adults. (See the PDQ summary on Childhood Brain Stem Glioma Treatment for more information.)
- Pineal astrocytic tumor: A pineal astrocytic tumor forms in tissue around the pineal gland and may be any grade. The pineal gland is a tiny organ in the brain that makes melatonin, a hormone that helps control the sleeping and waking cycle.
- Pilocytic astrocytoma (grade I): A pilocytic astrocytoma grows slowly in the brain or spinal cord. It may be in the form of a cyst and rarely spreads into nearby tissues. This type of tumor is most common in children and young adults and in people with neurofibromatosis type 1 (NF1). A pilocytic astrocytoma rarely causes death.
- Diffuse astrocytoma (grade II): A diffuse astrocytoma grows slowly, but often spreads into nearby tissues. Sometimes a diffuse astrocytoma progresses to a higher grade and becomes an anaplastic astrocytoma or a glioblastoma. A diffuse astrocytoma can form in any part of the brain but most often forms in the cerebrum. It is most common in young adults and in people with Li-Fraumeni syndrome. It is also called a low-grade diffuse astrocytoma.
- Anaplastic astrocytoma (grade III): An anaplastic astrocytoma grows quickly and spreads into nearby tissues. An anaplastic astrocytoma may progress to a higher grade and become a glioblastoma. An anaplastic astrocytoma forms most often in the cerebrum and is most common in adults. An anaplastic astrocytoma is also called a malignant astrocytoma or high-grade astrocytoma.
- Glioblastoma (grade IV): A glioblastoma grows and spreads very quickly. A glioblastoma forms most often in the cerebrum. This type of tumor is most common in adults. This type of tumor has a poor prognosis. It is also called glioblastoma multiforme.
Oligodendroglial Tumors
An oligodendroglial tumor begins in brain cells called oligodendrocytes, which help keep nerve cells healthy. Oligodendrocytes are a type of glial cell and are sometimes called a glioma. Grades of oligodendroglial tumors include the following:
- Oligodendroglioma (grade II): An oligodendroglioma grows and spreads slowly and the tumor cells look very much like normal cells. This type of tumor most often forms in the cerebrum. An oligodendroglioma is most common in adults.
- Anaplastic oligodendroglioma (grade III): An anaplastic oligodendroglioma grows quickly and the tumor cells look very different from normal cells. It may grow in one place or in many places throughout the brain. This type of cancer most often forms in the cerebrum.
Mixed Gliomas
A mixed glioma is a brain tumor that has two types of tumor cells in it — oligodendrocytes and astrocytes. This type of tumor most often forms in the cerebrum.
- Oligoastrocytoma (grade II): An oligoastrocytoma is a slow-growing tumor and the tumor cells don't look very different from normal cells.
- Anaplastic oligoastrocytoma (grade III): The tumor cells in an anaplastic oligoastrocytoma look very different from normal cells.
Ependymal Tumors
An ependymal tumor usually begins in cells that line the fluid-filled spaces in the brain and around the spinal cord. Ependymal cells are a type of glial cell and are sometimes called a glioma. Grades of ependymal tumors include the following:
- Ependymoma (grade I or II): A grade I or II ependymoma grows slowly and has cells that look very much like normal cells. There are two types of grade I ependymoma — myxopapillary ependymoma and subependymoma. These tumors are most common in adults. A grade II ependymoma grows in the ventricle and its connecting paths or in the spinal cord. It is most common in children and young adults and in people with neurofibromatosis type 2 (NF2). (See the PDQ summary on Childhood Ependymoma Treatment for more information.)
- Anaplastic ependymoma (grade III): An anaplastic ependymoma grows very quickly and has a poor prognosis.
Embryonal Cell Tumors: Medulloblastoma (Grade IV)
A medulloblastoma is a type of embryonal tumor. The tumor forms in brain cells when the fetus is beginning to develop. This type of brain tumor often begins in the cerebellum. The tumor may spread from the brain to the spine through the cerebrospinal fluid (CSF). A medulloblastoma occurs most often in children or young adults and in people with Turcot syndrome type 2 or nevoid basal cell carcinoma syndrome.
See the following PDQ summaries for more information on embryonal tumors in children:
Pineal Parenchymal Tumors
A pineal parenchymal tumor forms in parenchymal cells or pineocytes, which are the cells that make up most of the pineal gland. These tumors are different from pineal astrocytic tumors. Grades of pineal parenchymal tumors include the following:
- Pineocytomas (grade II): A pineocytoma is a slow-growing pineal tumor that occurs most often in adults.
- Pineoblastomas (grade IV): A pineoblastoma is a rare tumor that is very likely to spread. This type of tumor is most common in children. (See the PDQ summary on Childhood Central Nervous System Embryonal Tumors Treatment for more information.)
Meningeal Tumors
A meningeal tumor, also called a meningioma, forms in the meninges (thin layers of tissue that cover the brain and spinal cord). It can form from different types of brain or spinal cord cells. A meningioma is most common in adults. Types of meningeal tumors include the following:
- Meningioma (grade I): A grade I meningioma is the most common type of meningeal tumor. A grade I meningioma is a slow-growing, benign tumor that forms most often in the dura mater (the layer of tissue that covers the brain and is closest to the skull). It is most common in women.
- Meningioma (grade II and III): This is a rare, malignant meningeal tumor. It grows quickly and is likely to spread within the brain and spinal cord. A grade III meningioma is most common in men.
Germ Cell Tumors
A germ cell tumor forms in germ cells, which are the cells that develop into sperm in men or ova (eggs) in women. Germ cell tumors usually form in the center of the brain, near the pineal gland. Germ cell tumors can spread to other parts of the brain and spinal cord. There are different types of germ cell tumors. These include germinomas, teratomas, embryonal yolk sac carcinomas, and choriocarcinomas. Germ cell tumors can be either benign or malignant.
Most germ cell tumors occur in children and in people with Klinefelter syndrome. (See the PDQ summary on Childhood Brain and Spinal Cord Tumors Treatment Overview for more information.)
Tumors of the Sellar Region: Craniopharyngioma (Grade I) and Pituitary Tumor
A tumor of the sellar region begins in the center of the brain, just above the back of the nose. It can form from different types of brain or spinal cord cells.
- Craniopharyngioma (grade I): A craniopharyngioma is a rare tumor that usually forms just above the pituitary gland (a pea-sized organ at the bottom of the brain that controls other glands). The tumor may grow into nearby tissues, including the pituitary gland and optic nerves. This can affect many functions, including hormone-making and vision. These tumors occur in adults and children. (See the PDQ summary on Childhood Craniopharyngioma Treatment for more information on the treatment of children.)
- Pituitary tumor: See the PDQ summary on Pituitary Tumors Treatment for more information.
Other Adult Brain Tumors
There are many other types of adult brain tumors that are rare and are not discussed in this summary. See the PDQ health professional summary on Adult Brain Tumors Treatment for information about these other types of adult brain tumors:
Recurrent Brain Tumors
A recurrent brain tumor is a tumor that has recurred (come back) after it has been treated. Brain tumors often recur, sometimes many years after the first tumor. The tumor may recur at the same place in the brain or in other parts of the central nervous system.
The cause of most adult brain tumors is unknown.
Anything that increases your 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 doesn’t mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. There are few known risk factors for brain tumors. The following conditions may increase the risk of developing certain types of brain tumors:
- Being exposed to vinyl chloride may increase the risk of glioma.
- Past treatment with radiation therapy to the scalp or brain may increase the risk of meningioma.
- Infection with the Epstein-Barr virus, having AIDS (acquired immunodeficiency syndrome), or receiving an organ transplant may increase the risk of primary CNS lymphoma. (See the PDQ summary on Primary CNS Lymphoma for more information.)
- Having certain genetic syndromes may increase the risk of developing the following types of brain tumors:
- Neurofibromatosis type 1 or 2.
- Li-Fraumeni syndrome.
- Turcot syndrome type 1 and type 2.
- Klinefelter syndrome.
- Nevoid basal cell carcinoma syndrome.
The symptoms of adult brain and spinal cord tumors are not the same in every person.
The symptoms caused by a brain tumor depend on where the tumor formed in the brain, the functions controlled by that part of the brain, and the size of the tumor. Headaches and other symptoms may be caused by adult brain tumors. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:
- Morning headache or headache that goes away after vomiting.
- Frequent nausea and vomiting.
- Vision, hearing, and speech problems.
- Loss of balance and trouble walking.
- Weakness on one side of the body.
- Unusual sleepiness or change in activity level.
- Unusual changes in personality or behavior.
- Back pain or pain that spreads from the back towards the arms or legs.
- Weakness in the legs.
- Trouble walking.
Tests that examine the brain and spinal cord are used to detect (find) adult brain 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 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.
- Visual field exam: An exam to check a person’s field of vision (the total area in which objects can be seen). This test measures both central vision (how much a person can see when looking straight ahead) and peripheral vision (how much a person can see in all other directions while staring straight ahead). Any loss of vision may be a sign of a tumor that has damaged or pressed on the parts of the brain that affect eyesight.
- 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.
- Gene testing: A laboratory test in which a sample of blood or tissue is tested for changes in a chromosome that has been linked with a certain type of brain tumor.
- Lumbar puncture: A procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. The cerebrospinal fluid is viewed under a microscope by a pathologist to check for signs of cancer. This procedure is also called an LP or spinal tap.
- 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) with gadolinium: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of the brain and spinal cord. 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). Sometimes a procedure called magnetic resonance spectroscopy (MRS) is done during the MRI scan. An MRS is used to diagnose tumors, based on their chemical make-up.
- 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 of the brain. A small amount of a radioactive substance is injected into a vein or inhaled through the nose. As the substance travels through the blood, the camera rotates around the head and takes pictures of the brain. There will be increased blood flow and more chemical reactions (metabolism) 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.
- 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 brain. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
- Angiogram: A procedure to look at blood vessels and the flow of blood in the brain. 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.
Most adult brain tumors are diagnosed and removed in surgery.
If doctors think there may be a brain tumor, a biopsy may be done to remove a sample of tissue. For tumors in the brain, the biopsy is done by removing part of the skull and using a needle to remove the tissue sample. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor may remove as much tumor as safely possible during the same surgery. After the surgery, a pathologist checks the cancer cells to find out the type and grade of brain tumor. The grade of the tumor is based on how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. A CT scan or MRI may be used to find out if any cancer cells remain after surgery.
The following tests may be done on the tumor tissue that is removed:
- Immunohistochemistry study: A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of cancer tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer.
- Light and electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.
- Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options for primary brain tumors depend on the following:
- The type and grade of the tumor.
- Where the tumor is in the brain.
- Whether the tumor can be removed by surgery.
- Whether cancer cells remain after surgery.
- Whether there are certain changes in the chromosomes.
- Whether the cancer has just been diagnosed or has recurred (come back).
- The patient's general health.
- Whether the patient is younger than 60 years.
- Whether there are more than two tumors in the brain or spinal cord.
- Where in the brain or spinal cord the tumors are.
- How well the tumor responds to treatment.
- Whether the primary tumor continues to grow or spread.
Stages of Adult Brain Tumors
There is no standard staging system for adult brain tumors.
The extent or spread of cancer is usually described as stages. There is no standard staging system for brain tumors. Brain tumors that begin in the brain may spread to other parts of the brain and spinal cord, but they rarely spread to other parts of the body. Treatment of brain tumors is based on the type of cell in which the tumor began, where the tumor formed in the central nervous system, the amount of cancer left after surgery, and the grade of the tumor. Treatment of brain tumors that have spread to the brain from other parts of the body is based on the number of tumors in the brain.
Imaging tests may be repeated after surgery to help plan more treatment.
After the tumor is removed in surgery, some of the tests and procedures used to detect the brain tumor may be repeated to find out how much tumor is left.
Treatment Option Overview
There are different types of treatment for patients with adult brain tumors.
Different types of treatment are available for patients with adult brain 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. 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
Surgery is used to diagnose and treat adult brain tumors, as described in the General Information section of this summary.
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. One type of external radiation therapy is hyperfractionated radiation therapy, in which the total dose of radiation is divided into small doses 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 of tumor and where it is in the brain.
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. To treat brain tumors, a dissolving wafer may be used to deliver an anticancer drug directly to the brain tumor site after the tumor has been removed by surgery. The way the chemotherapy is given depends on the type of tumor and where it is in the brain.
New types of treatment are being tested in clinical trials.
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site.
Other types of radiation therapy
- Radiosensitizers: Drugs that make tumor cells more sensitive to radiation therapy. Combining radiation therapy with radiosensitizers may kill more tumor cells.
- Hyperfractionated radiation therapy: Hyperfractionated radiation therapy is radiation treatment in which the total dose of radiation is divided into small doses and the treatments are given more than once a day. Hyperfractionated radiation therapy is used as a standard treatment for a brain stem glioma.
- Accelerated-fraction radiation therapy: Radiation treatment in which the total dose of radiation is divided into small doses and the treatments are given more than once a day. The total dose of radiation is also given over a shorter period of time (fewer days) compared to standard radiation therapy.
- Intraoperative radiation therapy: A cancer treatment that uses high-energy x-rays to kill cancer cells during cancer surgery.
- Stereotactic radiosurgery: A type of radiation therapy that uses a rigid head frame attached to the skull to aim high-dose radiation beams directly at the tumors. This causes less damage to nearby healthy tissue. This is also called stereotaxic radiosurgery and radiation surgery. This procedure does not involve surgery.
Hyperthermia therapy
Hyperthermia therapy is a treatment in which body tissue is heated above normal temperature to damage and kill cancer cells or to make cancer cells more sensitive to the effects of radiation and certain anticancer drugs.
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.
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. 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 cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options by Type of Adult Brain Tumor
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.Astrocytic Tumors
Brain Stem Gliomas
Treatment of brain stem gliomas is radiation therapy, including hyperfractionated radiation therapy.Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with adult brain stem glioma. 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. General information about clinical trials is available from the NCI Web site.
Pineal Astrocytic Tumors
Treatment of pineal astrocytic tumors may include the following:- Surgery and radiation therapy, with or without chemotherapy.
- A clinical trial of external radiation therapy with or without radiosensitizers, intraoperative radiation therapy, or hyperthermia therapy.
- A clinical trial of external radiation therapy, followed by new anticancer drugs and biologic therapy.
Pilocytic Astrocytomas
Treatment of pilocytic astrocytoma is usually surgery with or without radiation therapy.Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with adult pilocytic astrocytoma. 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. General information about clinical trials is available from the NCI Web site.
Diffuse Astrocytomas
Treatment of diffuse astrocytoma may include the following:- Surgery with or without radiation therapy.
- A clinical trial of radiation therapy with or without chemotherapy for tumors that could not be completely removed by surgery.
- A clinical trial of radiation therapy given when the tumor progresses.
Anaplastic Astrocytomas
Treatment of anaplastic astrocytoma may include the following:- Surgery and radiation therapy, with or without chemotherapy.
- A clinical trial of external radiation therapy and one of the following:
- Accelerated-fraction radiation therapy.
- A clinical trial of external radiation therapy, followed by new anticancer drugs and biologic therapy.
- A clinical trial of chemotherapy combined with hyperfractionated radiation therapy or internal and external radiation therapy.
- A clinical trial of chemotherapy placed into the brain during surgery.
Glioblastoma
Treatment of glioblastoma may include the following:- Surgery and radiation therapy, with or without chemotherapy.
- Chemotherapy placed into the brain during surgery.
- Radiation therapy given at the same time as chemotherapy.
- A clinical trial of external radiation therapy and one of the following:
- Accelerated-fraction radiation therapy.
- A clinical trial of external radiation therapy, followed by new anticancer drugs and biologic therapy.
- A clinical trial of a new treatment.
Oligodendroglial Tumors
Treatment of oligodendrogliomas may include the following:- Surgery with or without radiation therapy.
- A clinical trial of radiation therapy with or without chemotherapy, for tumors that could not be completely removed by surgery.
- A clinical trial of chemotherapy.
- Surgery and radiation therapy, with or without chemotherapy.
- Radiation therapy with or without combination chemotherapy.
- A clinical trial of a new treatment.
Mixed Gliomas
Treatment of mixed gliomas may include the following:- Surgery and radiation therapy, with or without chemotherapy.
- Chemotherapy.
- A clinical trial of external radiation therapy and one of the following:
- A clinical trial of external radiation therapy, followed by new anticancer drugs and biologic therapy.
Ependymal Tumors
Treatment of grade I and grade II ependymomas is usually surgery with or without radiation therapy.Treatment of grade III anaplastic ependymoma may include the following:
- Surgery and radiation therapy.
- A clinical trial of chemotherapy before, during, or after radiation therapy.
Embryonal Cell Tumors: Medulloblastoma
Treatment of medulloblastomas may include the following:- Surgery and radiation therapy to the brain and spine.
- A clinical trial of surgery and radiation therapy to the brain and spine.
- A clinical trial of chemotherapy.
Pineal Parenchymal Tumors
Treatment of pineal parenchymal tumors may include the following:- Surgery and radiation therapy, for pineocytomas.
- Surgery, radiation therapy, and chemotherapy, for pineoblastomas.
- A clinical trial of external radiation therapy and one of the following:
- A clinical trial of external radiation therapy, followed by new anticancer drugs and biologic therapy.
Meningeal Tumors
Treatment of grade I meningiomas may include the following:- Surgery with or without radiation therapy.
- Radiation therapy for tumors that cannot be removed by surgery.
- Surgery and radiation therapy.
- A clinical trial of external radiation therapy and one of the following:
- A clinical trial of external radiation therapy, followed by new anticancer drugs and biologic therapy.
Germ Cell Tumors
Treatment of a germinoma is usually radiation therapy. For treatment of a teratoma that has not spread, surgery is used. There is no standard treatment for other types of germ cell tumors.Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with adult central nervous system 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. General information about clinical trials is available from the NCI Web site.
Tumors of the Sellar Region: Craniopharyngioma
Treatment of craniopharyngiomas may include the following:- Surgery to remove as much of the tumor as possible, followed by radiation therapy.
Recurrent Brain Tumors
Treatment of recurrent brain tumors may include the following:- Surgery with or without chemotherapy.
- Radiation therapy, if not used before, with or without chemotherapy.
- A second surgery followed by radiation therapy, if not used before, or chemotherapy.
- Chemotherapy.
- A clinical trial of a new anticancer drug with or without biologic therapy.
- A clinical trial of chemotherapy placed into the brain during surgery.
- A clinical trial of biologic therapy.
Metastatic Brain Tumors
Treatment of a single tumor that has spread to the brain from another part of the body is usually surgery followed by radiation therapy to the whole brain.Treatment of more than one tumor that has spread to the brain from another part of the body may include the following:
- Radiation therapy to the whole brain with or without stereotactic radiosurgery.
- Surgery, for tumors that are causing symptoms.
- Surgery to remove a tissue sample when the type of primary tumor is not known.
To Learn More About Adult Brain Tumors
For more information from the National Cancer Institute about adult brain tumors, see the following:For general cancer information and other resources from the National Cancer Institute, see the following:
Get More Information From NCI
Call 1-800-4-CANCERFor more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 8:00 a.m. to 8:00 p.m., Eastern Time. A trained Cancer Information Specialist is available to answer your questions.
Chat online
The NCI's LiveHelp® online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 8:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.
Write to us
For more information from the NCI, please write to this address:
- NCI Public Inquiries Office
- Suite 3036A
- 6116 Executive Boulevard, MSC8322
- Bethesda, MD 20892-8322
The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use the search box in the upper right corner of each Web page. The results for a wide range of search terms will include a list of "Best Bets," editorially chosen Web pages that are most closely related to the search term entered.
There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.
Find Publications
The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237).
Childhood Brain Stem Glioma
General Information About Childhood Brain Stem Glioma
Childhood brain stem glioma is a disease in which benign (noncancer) or malignant (cancer) cells form in the tissues of the brain stem.
The brain stem is the part of the brain connected to the spinal cord. It is located in the lowest part of the brain, just above the back of the neck. The brain stem is the part of the brain that controls breathing, heart rate, and nerves and muscles used in seeing, hearing, walking, talking, and eating. Most childhood brain stem gliomas are pontine gliomas, which form in a part of the brain stem called the pons. Brain tumors are the third most common type of cancer in children.
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This summary refers to the treatment of primary brain tumors (tumors that begin in the brain). Treatment for metastatic brain tumors, which are tumors formed by cancer cells that begin in other parts of the body and spread to the brain, is not discussed in this summary. Brain tumors can occur in both children and adults; however, treatment for children may be different than treatment for adults. See the following PDQ treatment summaries for more information:
The cause of most childhood brain tumors is unknown.
The symptoms of childhood brain stem glioma vary and often depend on the child’s age and where the tumor is located.
The following symptoms and others may be caused by a brain stem glioma. Other conditions may cause the same symptoms. A doctor should be consulted if any of these problems occur:
- Loss of balance and trouble walking.
- Vision and hearing problems.
- Morning headache or headache that goes away after vomiting.
- Nausea and vomiting.
- Unusual sleepiness or change in energy level.
Tests that examine the brain are used to detect (find) childhood brain stem glioma.
The following imaging tests may be used:
- 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) with gadolinium: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. 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).
Some childhood brain stem gliomas are diagnosed and removed in surgery.
If the tumor has not spread widely within the brain stem or has not been diagnosed by MRI, a biopsy may be done by removing part of the skull and using a needle to remove a sample of the brain tissue. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor will remove as much tumor as safely possible during the same surgery.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on:
- The type of brain stem glioma.
- Where the tumor is found in the brain and if it has spread within the brain stem.
- Whether or not the child has a condition called neurofibromatosis type 1.
- Whether the tumor has just been diagnosed or has recurred (come back).
Stages of Childhood Brain Stem Glioma
The plan for cancer treatment depends on whether the tumor is in one area of the brain or has spread throughout the brain.
Staging is the process used to find out how much cancer there is and if cancer has spread. It is important to know the stage in order to plan treatment.
There is no standard staging system for childhood brain stem glioma. Instead, the plan for cancer treatment depends on whether the tumor is diffuse (spread throughout the brain) or focal (in one area of the brain):
- Diffuse intrinsic pontine glioma is a tumor that has spread widely throughout the brain stem. A biopsy is usually not done for this type of brain stem glioma and it is not removed by surgery. A diffuse intrinsic pontine glioma is usually diagnosed using imaging studies.
- Focal or low-grade glioma is a tumor that is in one area of the brain stem. A biopsy may be done and the tumor removed during the same surgery.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
- Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
- Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.
The information from tests and procedures done to detect (find) childhood brain stem glioma is used to plan cancer treatment.
Some of the tests used to detect childhood brain stem glioma are repeated if the tumor is removed by surgery. (See the General Information section.) This is to find out how much tumor remains after surgery and plan further treatment.
Recurrent Childhood Brain Stem Glioma
Recurrent childhood brain stem glioma is a tumor that has recurred (come back) after it has been treated. If childhood brain stem glioma recurs, it may do so many years after initial treatment. The tumor may come back in the brain or in other parts of the central nervous system.Treatment Option Overview
There are different types of treatment for children with brain stem glioma.
Different types of treatment are available for children with brain stem glioma. 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 brain stem glioma should have their treatment planned by a team of health care providers who are experts in treating childhood brain tumors.
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 brain tumors and who specialize in certain areas of medicine. These may include the following specialists:
Childhood brain and spinal cord tumors may cause symptoms that begin before diagnosis and continue for months or years.
Childhood brain and spinal cord tumors may cause symptoms that continue for months or years. Symptoms caused by the tumor may begin before diagnosis. Symptoms caused by treatment may begin during or right after treatment.
Some cancer treatments cause side effects months or years after treatment has ended.
These are called late effects. Late effects may include the following:
- Physical problems.
- Changes in mood, feelings, thinking, learning, or memory.
- Second cancers (new types of cancer).
Five types of standard treatment are used:
Surgery
Surgery is used to diagnose and treat childhood brain stem glioma as discussed in the General Information section of this summary.
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.
Radiation therapy to the brain can affect growth and development in young children. Certain ways of giving radiation therapy can help keep radiation from damaging healthy tissue:
- Conformal radiation therapy is a type of radiation therapy that uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor. This allows a high dose of radiation to reach the tumor and causes less damage to normal tissue around the tumor.
- Hyperfractionated radiation therapy is radiation treatment in which the total dose of radiation is divided into small doses and the treatments are given more than once a day.
Several months after radiation therapy to the brain, imaging tests may show changes to the brain tissue. These changes may be caused by the radiation therapy or may mean the tumor is growing. It is important to be sure the tumor is growing before planning more treatment.
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 in 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.
Because radiation therapy to the brain can affect growth and brain development in young children, clinical trials are studying ways of using chemotherapy to delay or reduce the need for radiation therapy.
Cerebrospinal fluid diversion
Cerebrospinal fluid diversion is a method used to drain fluid that has built up around the brain and spinal cord. A shunt (long, thin tube) is placed in a ventricle (fluid-filled space) of the brain and threaded under the skin to another part of the body, usually the abdomen. The shunt carries excess fluid away from the brain so it may be absorbed elsewhere in the body.
Watchful waiting
Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change.
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 Web site.
Radiation therapy with 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. 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 cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Childhood Brain Stem Glioma
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.Untreated Childhood Brain Stem Glioma
Untreated childhood brain stem glioma is a tumor for which no treatment has been given. The child may have received drugs or treatment to relieve symptoms caused by the tumor.Standard treatment of diffuse intrinsic pontine glioma is usually radiation therapy.
Some of the treatments being studied in clinical trials for diffuse intrinsic pontine glioma include the following:
- Radiation therapy with a radiosensitizer.
- New anticancer drugs given at the same time as radiation therapy to the brain stem.
Standard treatment of focal or low-grade glioma may include the following:
- Surgery with or without radiation therapy, which may be followed by adjuvant chemotherapy.
- Cerebrospinal fluid diversion followed by watchful waiting.
Treatment of brain stem glioma in children with neurofibromatosis type 1 may be watchful waiting. The tumors are slow-growing in these children and may not need specific treatment for years.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with untreated childhood brain stem glioma. 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. General information about clinical trials is available from the NCI Web site.
Recurrent Childhood Brain Stem Glioma
Treatment of recurrent childhood brain stem glioma depends on the type of tumor, whether it comes back in the place in which it started or in another part of the brain, and the type of treatment previously given.Standard treatment of recurrent diffuse intrinsic pontine glioma is usually palliative therapy, to relieve symptoms and improve the patient's quality of life. The patient may also be treated in a clinical trial of a new treatment.
Treatment of recurrent focal or low-grade childhood brain stem glioma may include the following:
- A clinical trial of a new treatment.
To Learn More About Childhood Brain Tumors
For more information from the National Cancer Institute about childhood brain tumors, see the following:For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:
Get More Information From NCI
Call 1-800-4-CANCERFor more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 8:00 a.m. to 8:00 p.m., Eastern Time. A trained Cancer Information Specialist is available to answer your questions.
Chat online
The NCI's LiveHelp® online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 8:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.
Write to us
For more information from the NCI, please write to this address:
- NCI Public Inquiries Office
- Suite 3036A
- 6116 Executive Boulevard, MSC8322
- Bethesda, MD 20892-8322
The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use the search box in the upper right corner of each Web page. The results for a wide range of search terms will include a list of "Best Bets," editorially chosen Web pages that are most closely related to the search term entered.
There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.
Find Publications
The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237).
Changes to This Summary (01/06/2011)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.Images were added to this summary.
Last Modified: 2011-01-06
Childhood Cerebellar Astrocytoma
General Information About Childhood Astrocytomas
Childhood astrocytoma is a disease in which benign (noncancer) or malignant (cancer) cells form in the tissues of the brain.
Astrocytomas are tumors that start in star-shaped brain cells called astrocytes. An astrocyte is a type of glial cell. Glial cells hold nerve cells in place and help them work the way they should. There are several types of astrocytomas. They can form anywhere in the central nervous system (brain and spinal cord). Brain tumors are the third most common type of cancer in children.
The tumors may be benign (not cancer) or malignant (cancer). Benign brain tumors grow and press on nearby areas of the brain. They rarely spread into other tissues. Malignant brain tumors are likely to grow quickly and spread into other brain tissue. When a tumor grows into or presses on an area of the brain, it may stop that part of the brain from working the way it should. Both benign and malignant brain tumors can cause symptoms and need treatment.
This summary is about the treatment of primary brain tumors that begin in the glial cells in the brain. Information is included about the following tumors that form from glial cells:
- Astrocytomas.
Brain tumors can occur in both children and adults. However, treatment for children may be different than treatment for adults. (See the PDQ treatment summary on Adult Brain Tumors for more information.)
The central nervous system controls many important body functions.
Astrocytomas most commonly form in these parts of the central nervous system (CNS):
- Cerebrum: The largest part of the brain, at the top of the head. The cerebrum controls thinking, learning, problem-solving, speech, emotions, reading, writing, and voluntary movement.
- Cerebellum: The lower, back part of the brain (near the middle of the back of the head). The cerebellum controls movement, balance, and posture.
- Brain stem: The part that connects the brain to the spinal cord, in the lowest part of the brain (just above the back of the neck). The brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating.
- Hypothalamus: The area in the middle of the base of the brain that controls body temperature, hunger, and thirst.
- Visual pathway: The group of nerves that connect the eye with the brain.
- Spinal cord: The column of nerve tissue that runs from the brain stem down the center of the back. It is covered by three thin layers of tissue called membranes. The spinal cord and membranes are surrounded by the vertebrae (back bones). Spinal cord nerves carry messages between the brain and the rest of the body, such as a signal from the brain to cause muscles to move or from the skin to the brain for the sense of touch.
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The cause of most childhood brain tumors is not known.
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. Parents who think their child may be at risk should discuss this with their child's doctor. Possible risk factors for astrocytoma include:
- Past radiation therapy to the brain.
- Having certain genetic disorders, such as neurofibromatosis type 1 (NF1).
The symptoms of astrocytomas are not the same in every child.
Symptoms are different depending on the following:
- Where the tumor forms in the brain or spinal cord.
- The size of the tumor.
- How fast the tumor grows.
- The child's age and development.
- Morning headache or headache that goes away after vomiting.
- Nausea and vomiting.
- Vision, hearing, and speech problems.
- Loss of balance and trouble walking.
- Worsening handwriting or slow speech.
- Weakness or change in feeling on one side of the body.
- Unusual sleepiness or change in energy level.
- Change in personality or behavior.
- Weight loss or weight gain for no known reason.
- Increase in the size of the head (in infants).
Tests that examine the brain and spinal cord are used to detect (find) childhood astrocytomas.
The following tests and procedures may be used:
- Physical exam and history: An exam of the body to check general signs of health. This includes 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.
- 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 the brain and spinal cord. 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). Sometimes magnetic resonance spectroscopy (MRS) is done during the same MRI scan to look at the chemical makeup of the brain tissue.
- 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.
Childhood astrocytomas are diagnosed and removed in surgery.
If doctors think there may be an astrocytoma, a biopsy may be done to remove a sample of tissue. For tumors in the brain, the biopsy is done by removing part of the skull and using a needle to remove tissue. Sometimes, the needle is guided by a computer. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor may remove as much tumor as safely possible during the same surgery. Because it can be hard to tell the difference between types of brain tumors, you may want to have your child's tissue sample checked by a pathologist who has experience in diagnosing brain tumors.
The following tests may be done on the tissue that was removed:
- Immunohistochemistry study: A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of cancer tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer. An MIB-1 test is a type of immunohistochemistry study that checks tumor tissue for an antigen called MIB-1. This may show how fast a tumor is growing.
- Light and electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.
- Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
- Where the astrocytoma has formed in the CNS and if it has spread.
- Whether there are cancer cells left after surgery.
- The grade of astrocytoma.
- Whether the child has NF1.
- The child’s age.
- Whether the astrocytoma has just been diagnosed or has recurred (come back).
Stages of Childhood Astrocytomas
The grade of the tumor is used in place of a staging system to plan cancer treatment.
Staging is the process used to find out how much cancer there is and if cancer has spread. It is important to know the stage in order to plan treatment.
There is no standard staging system for childhood astrocytoma. Treatment is based on the grade of the tumor and whether it is untreated or recurrent (has come back after treatment). The grade of the tumor describes how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread.
The following grades are used:
Low-grade astrocytomas
Low-grade astrocytomas are slow-growing and rarely spread to other parts of the brain and spinal cord or other parts of the body. These include grade I (pilocytic, which form like a cyst and look almost like normal cells) and grade II (fibrillary, with cells that look long or slender like fibers) astrocytomas.
High-grade astrocytomas
High-grade astrocytomas are fast-growing and often spread within the brain and spinal cord. These include grade III (anaplastic or malignant) and grade IV (glioblastoma, which spreads the fastest) astrocytomas.
Childhood astrocytomas may form at more than one place in the brain, but they do not usually spread to other parts of the body. Children who have neurofibromatosis type 1 are more likely to have tumors in more than one place.
Tests are done to find out how much tumor remains after surgery and to plan further treatment.
Some of the tests used to detect astrocytomas are repeated after the tumor is removed. (See the General Information section.) This is to find out how much tumor remains after surgery and to plan further treatment. An MRI (magnetic resonance imaging) is done in the first 2 days after the surgery to see if there is any tumor left.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
- Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
- Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.
Recurrent Childhood Astrocytomas
A recurrent childhood astrocytoma is an astrocytoma that has recurred (come back) after it has been treated. The cancer may come back in the same place as the first tumor or in other parts of the body. High-grade astrocytomas often recur within 3 years.Treatment Option Overview
There are different types of treatment for patients with childhood astrocytoma.
Different types of treatment are available for children with astrocytomas. 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 astrocytomas should have their treatment planned by a team of health care providers who are experts in treating childhood brain tumors.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other healthcare providers who are experts in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists:
- Pediatric neurosurgeon.
Childhood brain tumors may cause symptoms that begin before diagnosis and continue for months or years.
Symptoms caused by the tumor may begin before diagnosis. These symptoms may continue for months or years. It is important to talk with your child's doctors about symptoms caused by the tumor, that may continue after treatment.
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.
- Changes in mood, feelings, thinking, learning, or memory.
- Second cancers (new types of cancer).
Six types of standard treatment are used:
Surgery
Surgery is used to diagnose and treat childhood astrocytoma as discussed in the General Information section of this summary. If cancer cells remain after surgery, further treatment depends on:
- Where the remaining cancer cells are.
- The grade of the tumor.
- Age of the child.
Cerebrospinal fluid diversion
Cerebrospinal fluid diversion is a method used to drain fluid that has built up around the brain and spinal cord. A shunt (long, thin tube) is placed in a ventricle (fluid-filled space) of the brain and threaded under the skin to another part of the body, usually the abdomen. The shunt carries excess fluid away from the brain so it may be absorbed elsewhere in the body.
Watchful waiting
Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change. Watchful waiting is often used for patients who have neurofibromatosis type1 or a tumor that is not growing and spreading.
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 location of cancer being treated.
Radiation therapy to the brain can affect growth and development in young children. Certain ways of giving radiation therapy can lessen the damage to healthy brain tissue:
- Conformal radiation therapy is a type of external radiation therapy. It uses a computer to make a 3-dimensional (3-D) picture of the tumor and shapes the radiation beams to fit the tumor. This allows a high dose of radiation to reach the tumor and causes less damage to normal tissue around the tumor.
- Stereotactic radiation therapy uses a rigid head frame attached to the skull to aim radiation directly to a tumor, causing less damage to normal tissue around 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.
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 the use of more than one anticancer drug. The way the chemotherapy is given depends on the type and location of the cancer being treated.
High-dose chemotherapy with stem cell transplant
High-dose chemotherapy with stem cell transplant is a way 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 reinfused stem cells grow into (and restore) the body's blood 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. (See the General Information section for a list of tests.) 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 astrocytoma has recurred (come back). If the tumor recurs in the brain, a biopsy may also be done to find out if it is made up of dead tumor cells or if new cancer cells are growing. These tests are sometimes called follow-up tests or check-ups. MRIs may be done regularly as follow-up to see if the tumor is growing back.
Treatment Options for Childhood Astrocytomas
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.Untreated Childhood Astrocytomas
Untreated low-grade astrocytomaWhen the tumor is first diagnosed, treatment for low-grade childhood astrocytoma depends on the location of the tumor and is usually surgery. An MRI is done after surgery to see if there is tumor remaining.
If the tumor was completely removed by surgery, more treatment may not be needed and the child is closely watched to see if symptoms appear or change. This is also called watchful waiting.
If there is tumor remaining after surgery, treatment may include the following:
- Watchful waiting.
- More surgery to remove the tumor.
- Radiation therapy, which may include conformal radiation therapy or stereotactic radiation therapy.
- Combination chemotherapy with or without radiation therapy.
- A clinical trial of conformal radiation therapy directed to the tumor only.
Children with neurofibromatosis type 1 (NF1) may not need treatment unless the tumor grows or symptoms, such as vision problems, appear.
Untreated high-grade astrocytoma
Treatment of untreated high-grade childhood astrocytoma may include the following:
- Surgery, chemotherapy, and radiation therapy to part or all of the brain.
- A clinical trial of chemotherapy with or without radiation therapy.
- A clinical trial of surgery followed by chemotherapy given at the same time as radiation therapy, followed by combination chemotherapy.
Recurrent Childhood Astrocytomas
Before more cancer treatment is given, imaging tests, biopsy, or surgery are done to be sure cancer is present and find out how much cancer there is.Recurrent low-grade astrocytoma
Treatment of recurrent low-grade childhood astrocytoma may include the following:
- Radiation therapy to the tumor only, if radiation therapy was not used when the tumor was first diagnosed. Conformal radiation therapy may be given.
- Surgery, chemotherapy, and/or radiation therapy, if the patient had only surgery when the tumor was first diagnosed.
- Chemotherapy if the tumor recurred after treatment with radiation therapy.
- Chemotherapy if the tumor recurred where it cannot be removed by surgery and the patient had radiation therapy when the tumor was first diagnosed.
- A clinical trial of a new treatment.
Treatment of recurrent high-grade childhood astrocytoma may include the following:
- Surgery and/or chemotherapy.
- A clinical trial of a new treatment.
To Learn More About Childhood Astrocytomas
For more information from the National Cancer Institute about Childhood Astrocytomas, see the following:For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:
Get More Information From NCI
Call 1-800-4-CANCERFor more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 8:00 a.m. to 8:00 p.m., Eastern Time. A trained Cancer Information Specialist is available to answer your questions.
Chat online
The NCI's LiveHelp® online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 8:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.
Write to us
For more information from the NCI, please write to this address:
- NCI Public Inquiries Office
- Suite 3036A
- 6116 Executive Boulevard, MSC8322
- Bethesda, MD 20892-8322
The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use the search box in the upper right corner of each Web page. The results for a wide range of search terms will include a list of "Best Bets," editorially chosen Web pages that are most closely related to the search term entered.
There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.
Find Publications
The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237).
Changes to This Summary (01/06/2011)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.Images were added to this summary.
2010-02-05 Last Modified: 2011-01-06
Childhood Ependymoma
General Information About Childhood Ependymoma
Childhood ependymoma is a disease in which malignant (cancer) cells form in the tissues of the brain and spinal cord.
The brain controls vital functions such as memory and learning, the senses (hearing, sight, smell, taste, and touch), and emotion. The spinal cord is made up of bundles of nerve fibers that connect the brain with nerves in most parts of the body.
About 1 in 11 childhood brain tumors are ependymomas. Although cancer is rare in children, brain tumors are the most common type of childhood cancer other than leukemia and lymphoma.
This summary refers to the treatment of primary brain tumors (tumors that begin in the brain). Treatment of metastatic brain tumors, which are tumors formed by cancer cells that begin in other parts of the body and spread to the brain, is not discussed in this summary.
There are many different types of brain tumors. Brain tumors can occur in both children and adults; however, treatment for children may be different than treatment for adults. See the following PDQ summaries for more information:
The central nervous system controls many important body functions.
Ependymomas most commonly form in these parts of the central nervous system (CNS):
- Cerebrum: The largest part of the brain, at the top of the head. The cerebrum controls thinking, learning, problem-solving, speech, emotions, reading, writing, and voluntary movement.
- Cerebellum: The lower, back part of the brain (near the middle of the back of the head). The cerebellum controls movement, balance, and posture.
- Brain stem: The part that connects the brain to the spinal cord, in the lowest part of the brain (just above the back of the neck). The brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating.
- Spinal cord: The column of nerve tissue that runs from the brain stem down the center of the back. It is covered by three thin layers of tissue called membranes. The spinal cord and membranes are surrounded by the vertebrae (back bones). Spinal cord nerves carry messages between the brain and the rest of the body, such as a signal from the brain to cause muscles to move or from the skin to the brain for the sense of touch.
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The cause of most childhood brain tumors is unknown.
The symptoms of childhood ependymoma vary and often depend on the child’s age and where the tumor is located.
The following symptoms and others may be caused by childhood ependymoma. Other conditions may cause the same symptoms. A doctor should be consulted if any of these problems occur:
- Frequent headaches.
- Loss of balance or trouble walking.
Tests that examine the brain and spinal cord are used to detect (find) childhood ependymoma.
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 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.
- 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) with gadolinium: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain and spinal cord. A substance called gadolinium is injected into the patient through 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).
- Lumbar puncture: A procedure used to collect cerebrospinal fluid from the spinal column to check for cancer cells. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.
Childhood ependymoma is diagnosed and removed in surgery.
If a brain tumor is suspected, a biopsy is done by removing part of the skull and using a needle to remove a sample of the brain tissue. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor will remove as much tumor as safely possible during the same surgery. An MRI may be done after the tumor is removed to find out how much tumor remains.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on:
- Whether cancer cells remain after surgery.
- The type of ependymoma and whether it begins in the brain or in the spinal cord.
- The age of the child when the tumor is diagnosed.
- Whether the cancer has spread to other parts of the central nervous system, such as the meninges (membranes covering the brain) and the spinal cord.
- Whether the cancer has spread to other parts of the body, such as the bone or lung.
- Whether the tumor has just been diagnosed or has recurred (come back).
Stages of Childhood Ependymoma
The area where the tumor is found and the child’s age are used in place of a staging system to plan cancer treatment.
Staging is the process used to find out how much cancer there is and if cancer has spread. It is important to know the stage in order to plan treatment.
There is no standard staging system for childhood ependymoma. Instead, the plan for cancer treatment after surgery depends on the following:
- Whether any cancer cells remain after surgery.
- Whether the cancer has spread to other parts of the brain or spinal cord.
- The age of the child.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
- Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
- Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.
The information from tests and procedures done to detect (find) childhood ependymoma is used to plan cancer treatment.
Some of the tests used to detect childhood ependymoma are repeated after the tumor is removed by surgery. (See the General Information section.) This is to find out how much tumor remains after surgery. Another procedure that may be done to find out if cancer has spread is a lumbar puncture. A lumbar puncture is a procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.
Recurrent Childhood Ependymoma
Recurrent childhood ependymoma is a tumor that has recurred (come back) after it has been treated. Childhood ependymoma commonly recurs, usually at the original cancer site. The tumor may come back as long as 15 years or more after initial treatment.Treatment Option Overview
There are different types of treatment for children with ependymoma.
Different types of treatment are available for children with ependymoma. 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 ependymoma should have their treatment planned by a team of health care providers who are experts in treating childhood brain tumors.
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 brain tumors and who specialize in certain areas of medicine. These may include the following specialists:
- Pediatric neurosurgeon.
Childhood brain and spinal cord tumors may cause symptoms that begin before diagnosis and continue for months or years.
Childhood brain and spinal cord tumors may cause symptoms that continue for months or years. Symptoms caused by the tumor may begin before diagnosis. Symptoms caused by treatment may begin during or right after treatment.
Some cancer treatments cause side effects months or years after treatment has ended.
These 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).
Three types of standard treatment are used:
Surgery
Surgery is used to diagnose and treat childhood ependymoma as described in the General Information section of this summary.
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. Childhood ependymoma may be treated with fractionated radiation therapy, which divides the total dose of radiation into several smaller, equal doses delivered over a period of days.
Certain ways of giving radiation therapy can help keep radiation away from healthy tissue:
- Conformal radiation therapy uses a computer to create a 3-D picture of the tumor. The radiation beams are shaped to fit the tumor.
- Proton-beam therapy is a type of high-energy, external radiation therapy that uses streams of protons (small, positively-charged particles of matter) to kill tumor cells.
- Stereotactic radiation therapy uses a head frame attached to the skull to aim radiation beams directly at the tumor.
Damage to the brain in young children treated for ependymoma is not always due to the effects of radiation therapy. For example, when hydrocephalus (abnormal buildup of fluid in the brain) is found at diagnosis, it is linked with lower intelligence test scores following surgery and before radiation 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.
Watchful waiting
Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change.
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. 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 cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Childhood Ependymoma
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.Newly Diagnosed Childhood Ependymoma
Newly diagnosed childhood ependymoma is a tumor that has not been treated. The patient may have received drugs or treatment, however, to relieve symptoms caused by the tumor.
Initial treatment for newly diagnosed childhood ependymoma is usually surgery, with or without additional treatment.
After surgery, treatment depends on the age of the child, the amount of tumor that was removed, and whether cancer cells have spread to other parts of the central nervous system.
When the tumor is completely removed by surgery and cancer cells have not spread within the central nervous system, treatment may include the following:
- Radiation therapy to the tumor bed (where the tumor was before it was removed), for children aged 3 years or older.
- Chemotherapy, for children younger than 3 years.
- A clinical trial of watchful waiting, for children aged 1 year or older.
- A clinical trial of chemotherapy with or without radiation therapy.
When a part of the tumor remains after surgery, but cancer cells have not spread within the central nervous system, treatment may include the following:
- Radiation therapy to the tumor bed, for children aged 3 years or older.
- A clinical trial of chemotherapy followed by second-look surgery and conformal radiation therapy.
When cancer cells have spread within the central nervous system, treatment may include the following:
- Radiation therapy to the whole brain and spine.
- A clinical trial of radiation therapy and chemotherapy.
Recurrent Childhood Ependymoma
Treatment of recurrent childhood ependymomas may include the following:- Radiation therapy, including stereotactic radiation therapy.
- Chemotherapy, if chemotherapy was not used when the tumor was first diagnosed.
- A clinical trial of a new treatment.
To Learn More About Childhood Brain Tumors
For more information from the National Cancer Institute about childhood brain tumors, see the followingFor more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:
Get More Information From NCI
Call 1-800-4-CANCERFor more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 8:00 a.m. to 8:00 p.m., Eastern Time. A trained Cancer Information Specialist is available to answer your questions.
Chat online
The NCI's LiveHelp® online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 8:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.
Write to us
For more information from the NCI, please write to this address:
- NCI Public Inquiries Office
- Suite 3036A
- 6116 Executive Boulevard, MSC8322
- Bethesda, MD 20892-8322
The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use the search box in the upper right corner of each Web page. The results for a wide range of search terms will include a list of "Best Bets," editorially chosen Web pages that are most closely related to the search term entered.
There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.
Find Publications
The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237).
Changes to This Summary (01/06/2011)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.Images were added to this summary.
Last Modified: 2011-01-06
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