Ependymoma – Childhood

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:

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

The central nervous system controls many important body functions.
Ependymomas most commonly form in these parts of the central nervous system (CNS):

• Cerebrum: The largest part of the brain, at the top of the head. The cerebrum controls thinking, learning, problem-solving, speech, emotions, reading, writing, and voluntary movement.
• Cerebellum: The lower, back part of the brain (near the middle of the back of the head). The cerebellum controls movement, balance, and posture.
• Brain stem: The part that connects the brain to the spinal cord, in the lowest part of the brain (just above the back of the neck). The brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating.
• Spinal cord: The column of nerve tissue that runs from the brain stem down the center of the back. It is covered by three thin layers of tissue called membranes. The spinal cord and membranes are surrounded by the vertebrae (back bones). Spinal cord nerves carry messages between the brain and the rest of the body, such as a signal from the brain to cause muscles to move or from the skin to the brain for the sense of touch.

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:

• 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:

• Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
• Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
• 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.

Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) and treatment options depend on:

• Whether cancer cells remain after surgery.
• The type of ependymoma and whether it begins in the brain or in the spinal cord.
• The age of the child when the tumor is diagnosed.
• Whether the cancer has spread to other parts of the central nervous system, such as the (membranes covering the brain) and the spinal cord.
• Whether the cancer has spread to other parts of the body, such as the bone or lung.
• Whether the tumor has just been diagnosed or has recurred (come back).

Treatment Options for Childhood Ependymoma
Newly Diagnosed Childhood Ependymoma
Recurrent 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.
• Chemotherapy, for children younger than 3 years.
• 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.

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.