Childhood Ependymoma Treatment (PDQ®)–Patient Version

General Information About Childhood Ependymoma

KEY POINTS

Childhood ependymoma is a disease in which malignant (cancer) cells form in the tissues of the brain and spinal cord.
There are different types of ependymomas.
The part of the brain that is affected depends on where the ependymoma forms.
The cause of most childhood brain tumors is unknown.
The signs and symptoms of childhood ependymoma are not the same in every child.
Tests that examine the brain and spinal cord are used to detect (find) childhood ependymoma.
Childhood ependymoma is diagnosed and removed in surgery.
Certain factors affect prognosis (chance of recovery) and treatment options.

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:

Subependymoma (WHO Grade I).
Myxopapillary ependymoma (WHO Grade I).
Ependymoma (WHO Grade II).
RELA fusion–positive ependymoma (WHO Grade II or Grade III with change in the RELAgene).
Anaplastic ependymoma (WHO Grade III).

The grade of a tumor describes how abnormal the cancer cells look under a microscopeand 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.

Drawing 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). — 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:

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 message from the brain to cause muscles to move or a message from the skin to the brain to feel touch.

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:

The child's age.
Where the tumor has formed.

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:

Frequent headaches.
Seizures.
Nausea and vomiting.
Pain or stiffness in the neck.
Loss of balance or trouble walking.
Weakness in the legs.
Blurry vision.
Back pain.
A change in bowel function.
Trouble urinating.
Confusion or irritability.

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.
MRI (magnetic resonance imaging) with gadolinium : 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 a veinand travels through the bloodstream. 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 (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of fluid. The sample of CSF is checked under a microscope for signs of tumor cells. The sample may also be checked for the amounts of protein and glucose. A higher than normal amount of protein or lower than normal amount of glucose may be a sign of a tumor. This procedure is also called an LP or spinal tap.
ENLARGE
Lumbar puncture. A patient lies in a curled position on a table. After a small area on the lower back is numbed, a spinal needle (a long, thin needle) is inserted into the lower part of the spinal column to remove cerebrospinal fluid (CSF, shown in blue). The fluid may be sent to a laboratory for testing.

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.

Drawing of a craniotomy showing a section of the scalp that has been pulled back to remove a piece of the skull; the dura covering the brain has been opened to expose the brain. The layer of muscle under the scalp is also shown. — 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:

Immunohistochemistry : A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between brain stem glioma and other brain tumors.

An MRI is often done after the tumor is removed to find out whether any tumor remains.