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 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:
Tests that examine the brain and spinal cord are used to detect (find) childhood ependymoma.
The following tests and procedures may be used:
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.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on:
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:
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
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 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.
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:
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:
Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information).
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.
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.
Certain ways of giving radiation therapy can help keep radiation away from healthy tissue:
Radiation therapy to the brain can affect growth and development in young children and is not standard treatment for children younger than 3 years. For this reason, conformal radiation therapy that limits damage to healthy brain tissue is being studied in infants and children with ependymoma.
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 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.
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI Web site.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the 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 clinical trials database.
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.
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 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:
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:
When cancer cells have spread within the central nervous system, treatment may include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with newly diagnosed childhood ependymoma.
Standard treatment of recurrent childhood ependymomas may include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood ependymoma.
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:
Call 1-800-4-CANCER
For 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 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.
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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), TTY at 1-800-332-8615.
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.
Changes were made to this summary to match those made to the health professional version.
Last Modified: 2008-07-03
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