Ependymoma (Childhood)

General Information
What is 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.

Approximately 10% of all 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 for metastatic brain tumors, which are secondary 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. (Refer to Adult Brain Tumor Treatment for more information.)

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.
-Frequent nausea and vomiting.
-Loss of balance or difficulty walking.

Tests that examine the brain and spinal cord are used to detect (find) and diagnose childhood ependymoma.

These tests may include the following:

CT scan (CAT scan): A CT scan creates a series of detailed pictures of areas inside the body, taken from different angles. The pictures are created by a computer linked to an x-ray machine. This test is also called computed tomography, computerized tomography, or computerized axial tomography.

MRI (magnetic resonance imaging): A procedure in which a magnet linked to a computer is used to create detailed pictures of areas inside the body. This test is also called nuclear magnetic resonance imaging (NMRI).

Biopsy: After a brain tumor has been found, a biopsy is done by making a small hole in the skull and using a needle to remove a sample of the tumor. The tissue is then examined under a microscope by a pathologist to see what type of tumor it is. If it is an ependymoma, the doctor will attempt to remove as much of the tumor as possible. The pathologist will also determine the grade of the tumor. The cells of higher grade tumors look more abnormal, grow faster, and are more likely to spread than the cells of lower grade tumors.

Certain factors affect prognosis (chance of recovery). The most important factors that affect prognosis (chance of recovery) are thought to be the amount of tumor removed during surgery and the age of the child when the tumor was found.

Stages of Childhood Ependymoma

Staging is the process used to find out how far the cancer has spread. There is no standard staging system for childhood ependymoma. The tumors are described by grade and by where they are located in the central nervous system (brain and spinal cord).

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. 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:

Rehabilitation specialist.
Radiation oncologist.
Medical oncologist.

Three types of standard treatment are used:

Surgery is used to treat childhood ependymomas. Once a childhood ependymoma has been diagnosed, the child's doctor will remove as much of the tumor as safely possible during a craniotomy (an operation in which an opening is made in the skull). If the ependymoma is in a place where it cannot be removed, surgery may be limited to a biopsy of the tumor.

After surgery, tests will be done to find out if the ependymoma has spread to other parts of the central nervous system (brain and spinal cord). These tests include:

-Magnetic resonance imaging of the total spine: A magnet linked to a computer is used to create detailed pictures of the spine. This test is also called MRI or nuclear magnetic resonance imaging (NMRI).

-Lumbar puncture: A procedure in which a needle is put into the lower part of the spinal column to collect cerebrospinal fluid. This procedure is also called a spinal tap.

Radiation therapy
Radiation therapy is the use of x-rays or other types of radiation to kill cancer cells and shrink tumors. Radiation therapy may use external radiation (using a machine outside the body) or internal radiation. Internal radiation involves putting radioisotopes (materials that produce radiation) through thin plastic tubes into the area where cancer cells are found. External radiation is usually used to treat childhood ependymoma. Radiation therapy can affect growth and development in young children and is not standard treatment for children younger than 3 years of age.

Chemotherapy is the use of drugs to kill cancer cells. Chemotherapy may be taken by mouth, or it may be put into the body by inserting a needle into a vein or muscle. Either type of chemotherapy is called systemic treatment because the drugs enter the bloodstream, travel through the body, and can kill cancer cells throughout the body.

Other types of treatment are being tested in clinical trials.

Treatment Options For 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.
-A clinical trial of radiation therapy and chemotherapy.

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:

-Additional surgery.
-Radiation therapy to the tumor.
-A clinical trial of radiation therapy and chemotherapy.

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.

Treatment after surgery for children younger than 3 years of age may include the following:

-A clinical trial of chemotherapy to delay the need for radiation therapy.
-A clinical trial of radiation therapy.

This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied.

Treatment Options for Recurrent Childhood Ependymoma
Treatment of recurrent childhood ependymomas may include the following:

-Radiation therapy.
-A clinical trial of a new therapy.

The information on this page was obtained from the National Cancer Institute. The National Cancer Institute provides accurate, up-to-date information on many types of cancer, information on clinical trials, resources for people dealing with cancer, and information for researchers and health professionals.

The National Cancer Institute is in no way affiliated with the Mary Stolfa Cancer Foundation.

The information on this web site is provided for general information only. It is not intended as medical advice, and should not be relied upon as a substitute for consultations with qualified health professionals who are familiar with your individual medical needs. The MSCF disclaims all obligations and liabilities for damages arising from the use or attempted use of the information, including but not limited to direct, indirect, special, and consequential damages, attorneys' and experts' fees and court costs. Any use of the information will be at the risk of the user.

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