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This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http://cancer.gov or call 1-800-4-CANCER.
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, emotion, and the senses (hearing, sight, smell, taste, and touch). The spinal cord is made up of bundles of nerve fibers that connect the brain with nerves in most parts of the body.
Ependymomas form from ependymal cells that line the ventricles and passageways in the brain and the spinal cord. Ependymal cells make cerebrospinal fluid (CSF).
This summary is about the treatment of primary brain tumors (tumors that begin in the brain). Treatment of metastatic brain tumors, which are tumors 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 is different than treatment for adults. See the following PDQ summaries for more information:
There are different types of ependymomas.
The World Health Organization (WHO) groups ependymal tumors into five main subtypes:
The grade of a tumor describes how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. Low-grade (Grade I) cancer cells look more like normal cells than high-grade cancer cells (Grade II and III). They also tend to grow and spread more slowly than Grade II and III cancer cells.
The part of the brain that is affected depends on where the ependymoma forms.
Ependymomas can form anywhere in the fluid -filled ventricles and passageways in the brain and spinal cord. Most ependymomas form in the fourth ventricle and affect the cerebellum and the brain stem. Anatomy of the inside of the brain showing the lateral ventricle, third ventricle, fourth ventricle, and the passageways between the ventricles (with cerebrospinal fluid shown in blue). Other parts of the brain shown include the cerebrum, cerebellum, spinal cord, and brain stem (pons and medulla).
Once an ependymoma forms, areas of the brain that may be affected include:
The cause of most childhood brain tumors is unknown.
The signs and symptoms of childhood ependymoma are not the same in every child.
Signs and symptoms depend on the following:
Signs and symptoms may be caused by childhood ependymoma or by other conditions. Check with your child's doctor if your child has any of the following:
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 the diagnostic tests show there may be a brain tumor, 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.
Craniotomy: An opening is made in the skull and a piece of the skull is removed to show part of the brain.
The following test may be done on the tissue that was removed:
An MRI is often done after the tumor is removed to find out whether any tumor remains.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on:
Prognosis also depends on the type and dose of radiation therapy that is given.
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. There is no standard staging system for childhood ependymoma. Treatment is based on where the cancer is in the body and the age of the child.
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.
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 signs or symptoms that begin before the cancer is diagnosed and continue for months or years.
Childhood brain and spinal cord tumors may cause signs or symptoms that continue for months or years. Signs or symptoms caused by the tumor may begin before diagnosis. Signs or symptoms caused by treatment may begin during or right after treatment.
Treatment for childhood ependymoma may cause side effects.
For information about side effects that begin during treatment for cancer, see our Side Effects page.
Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:
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).
Four types of standard treatment are used:
If the results of diagnostic tests show there may be a brain tumor, 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 check for cancer cells. If cancer cells are found, the doctor will remove as much tumor as safely possible during the same surgery. Craniotomy: An opening is made in the skull and a piece of the skull is removed to show part of the brain.
An MRI is often done after the tumor is removed to find out whether any tumor remains. If tumor remains, a second surgery to remove as much of the remaining tumor as possible may be done.
Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment to lower the risk that the cancer will come back after surgery is called adjuvant 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:
The way the radiation therapy is given depends on the type of cancer being treated. External radiation therapy is used to treat childhood ependymoma.
Children younger than 3 years who receive radiation therapy to the brain have a higher risk of problems with growth and development than older children. 3-D conformal radiation therapy and proton-beam therapy are being studied in children younger than 3 years to see if the effects of radiation on growth and development are lessened.
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 of cancer being treated.
Observation is closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change. Observation may be used to treat a child with a subependymoma who has no symptoms and whose tumor is found while treating another condition.
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.
Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do.
Targeted therapy is being studied for the treatment of childhood ependymoma that has recurred (come back).
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. Information about clinical trials supported by NCI can be found on NCI's clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Follow-up tests for childhood ependymoma include an MRI (magnetic resonance imaging) of the brain and spinal cord every 3 months for the first 1 or 2 years after treatment. After 2 years, MRIs may be done every 6 months for the next 3 years.
For information about the treatments listed below, see the Treatment Option Overview section.
Newly Diagnosed Childhood Ependymoma
A child with a newly diagnosed ependymoma has not had treatment for the tumor. The child may have had treatment to relieve signs or symptoms caused by the tumor.
Treatment of newly diagnosed subependymoma (WHO Grade I) is:
Treatment of newly diagnosed myxopapillary ependymoma (WHO Grade I) is:
Childhood ependymoma, anaplastic ependymoma, orRELAfusion-positive ependymoma
Treatment of newly diagnosed childhood ependymoma (WHO Grade II), anaplastic ependymoma (WHO Grade III), or RELA fusion-positive ependymoma (WHO Grade II or Grade III) is:
After surgery, the plan for further treatment depends on the following:
When the tumor is completely removed and cancer cells have not spread, treatment may include the following:
When part of the tumor remains after surgery, but cancer cells have not spread, treatment may include the following:
When cancer cells have spread within the brain and spinal cord, treatment may include the following:
Treatment for children younger than 3 years of age may include the following:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Recurrent Childhood Ependymoma
Treatment of recurrent childhood ependymoma may include the following:
For more information about childhood brain tumors, see the following:
For more childhood cancer information and other general cancer resources, see the following:
Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government's center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood ependymoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Date Last Modified") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
Clinical Trial Information
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." 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.
Clinical trials are listed in PDQ and can be found online at NCI's website. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237).
Permission to Use This Summary
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as "NCI's PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary]."
The best way to cite this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Ependymoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/brain/patient/child-ependymoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389185]
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Last Revised: 2018-03-16
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