What is an ependymoma?

An ependymoma is a tumor (abnormal growth or mass of cells) that begins in the ependymal cells in the brain and spinal cord that line the passageways where the fluid (cerebrospinal fluid) that nourishes the brain flows.

Pediatric ependymomas usually appear in the brain, most commonly in the posterior fossa (back part of the brain). In rare cases, an ependymoma can arise in the spinal cord.

Ependymomas account for 5 to 10 percent of pediatric brain tumors and occur equally in boys and girls. These types of tumors are the third most common brain tumor in children.

Although rarely occurring in the spinal cord, ependymomas do account for about 25 percent of all spinal cord tumors. Most patients with tumors of the spinal cord are older than 12.

What causes an ependymoma? Who is affected?

Ependymomas can occur at any age, but most often occurs in infants and young children. The cause of ependymoma is not known.

In children 0-14 years, ependymoma accounted for 5.7% of all tumors diagnosed. Approximately 185 children are diagnosed annually in the United States.

In children 15-19 years, ependymoma accounted for 4% of all tumors diagnosed. Approximately 50 teenagers are diagnosed annually in the United States.

The outlook for patients diagnosed with an ependymoma depends on a variety of factors, including location of the tumor, how much of the tumor was removed, if it has metastasized (spread), if it has recurred, and more.
– With complete surgical resection of the tumor, there is about a 65% survival rate.
– Metastatic, progressive, or recurrent ependymomas are more difficult to treat.

Where are the symptoms of ependymoma?

Symptoms often include headaches, hydrocephalus (the buildup of fluid in the brain that can result in increased head size in infants, or headaches and vomiting in older children), morning nausea or vomiting, lethargy, irritability, problems eating or walking.

How is a ependymoma treated?

Treatment almost always begins with surgery as the first step. The goal is to remove as much of the tumor as possible. Sometimes the location of the tumor or other factors may make it impossible for the neurosurgeons to remove completely.

If a tumor causes blockage of cerebral spinal fluid flow, a procedure may be performed to relieve symptoms of hydrocephalus, the buildup of fluid inside the skull.

Ependymoma treatment depends on the site of the tumor and if it is recurrent. Within the brain and posterior fossa, outcomes are best when the tumor is completely removed during surgery and is typically followed by high dose focal radiation therapy.

Radiation therapy uses high-energy waves or other types of radiation to kill cancer cells or stop them from growing.

Chemotherapy uses powerful medication to kill cancer cells or stop them from growing and making more cancer cells. Chemo is injected or given orally so that it can travel throughout the body. Combination therapy is when more than one chemotherapy drug is used at one time.