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. Refer to the following PDQ summaries for more information:

• Childhood Brain and Spinal Cord Tumors Treatment
• Adult Brain Tumors Treatment

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.

These symptoms may be caused by childhood ependymoma or other conditions. A doctor should be consulted if any of the following problems occur:

• Frequent headaches.
• Seizures.
• Frequent nausea and vomiting.
• 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:

• 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 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).

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).

The prognosis (chance of recovery) depends on:

• Amount of tumor removed during surgery.
• Tumor histology (how the tumor cells look under a microscope).
• The age of the child when the tumor was found.

Stages of Childhood Ependymoma

After childhood ependymoma has been removed, tests are done to find out if there is tumor remaining.

The extent or spread of cancer is usually described as stages. For childhood ependymoma, tumors are described by grade and by where they are located in the central nervous system (brain and spinal cord). The grade of the tumor refers to how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. It is important to know the grade of the tumor and if there were any cancer cells remaining after surgery in order to plan treatment. The following tests and procedures may be used:

• 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 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. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.

There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

• Through tissue. Cancer invades the surrounding normal tissue.
• 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.

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.

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 doctors with expertise in treating childhood brain tumors.

Your child’s treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist may refer you to other pediatric doctors who have experience and expertise in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists:

• Neurosurgeon.
• Neurologist.
• Neuropathologist.
• Neuroradiologist.
• Rehabilitation specialist.
• Radiation oncologist.
• Medical oncologist.
• Endocrinologist.
• Psychologist.

Some cancer treatments cause side effects that continue or appear years after cancer treatment has ended. These are called late effects. Late effects of cancer treatment may include physical problems; changes in mood, feelings, thinking, learning or memory; and having second cancers (new types of cancer). Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments. Refer to 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. 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:

• Conformal radiation therapy uses a computer to create a 3-D picture of the tumor. The radiation beams are shaped to fit the tumor.
• Stereotactic radiation therapy uses a head frame attached to the skull to aim radiation beams directly at the tumor.

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 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.

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 will 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.

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.
• A clinical trial of watchful waiting, for children aged 1 year or older.
• A clinical trial of conformal radiation therapy.
• 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:

• Second-look surgery.
• 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, for children aged 1 year or older.

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.

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.

Recurrent Childhood Ependymoma

Standard treatment of recurrent childhood ependymomas may include the following:

• Surgery.
• Radiation therapy, including stereotactic radiation therapy with or without chemotherapy.
• Chemotherapy with or without radiation therapy.

New treatments for recurrent childhood ependymomas are being studied in clinical trials. Information about clinical trials is available from the NCI Web site.

Check for clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood ependymoma.

To Learn More About Childhood Brain Tumors

For more information from the National Cancer Institute about childhood brain tumors, see the following:

• What You Need to Know About™ Brain Tumors
• Pediatric Brain Tumor Consortium (PBTC)

For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:

• What You Need to Know About™ Cancer - An Overview
• Childhood Cancers
• CureSearch - National Childhood Cancer Foundation Children’s Oncology Group
• Late Effects of Treatment for Childhood Cancer
• Young People with Cancer: A Handbook for Parents
• Care for Children and Adolescents with Cancer: Questions and Answers
• Understanding Cancer Series: Cancer
• Staging: Questions and Answers
• Coping with Cancer
• Support and Resources
• Cancer Library
• Information for Survivors/Caregivers/Advocates

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Changes to This Summary (04/04/2008)

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.

Several enhancements have been made to this summary to better explain certain medical concepts and to help readers find information about clinical trials. The following changes were made:

• Text describing the way cancer spreads in the body was added to the Stages section.
• Information about patients taking part in clinical trials and about follow-up tests was added to the Treatment Option Overview section.
• Links to ongoing clinical trials listed in NCI's PDQ Cancer Clinical Trials Registry were added to the Treatment Options section.
• A new section called "To Learn More" was added. It includes links to more information about this type of cancer and about cancer in general.
• The "Get More Information from NCI" section (originally called "To Learn More") was revised.