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. Refer to the following PDQ summaries for more information:


Childhood Brain 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 the 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.




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. Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.



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.