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What is an ependymoma?

An ependymoma is a tumor that arises from cells that are found lining the ventricular system (areas of the brain or spinal cord where spinal fluid is found). These tumors can form in any of the ventricles in the brain or spinal cord, but most ependymomas arise in the fourth ventricle and affect the cerebellum and the brain stem.

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

What are the symptoms of epedymomas?

Brain tumors can cause a variety of symptoms in children depending on their size and location. Keep in mind that the symptoms of a brain tumor may resemble other more common conditions or medical problems. It is important to consult your child's physician if you have concerns.

Ependymoma symptoms are most often associated with increased pressure in the brain and can include:

  • headache — generally upon awakening in the morning
  • hydrocephalus
  • nausea and vomiting
  • lethargy
  • irritability
  • problems eating or walking

How we care for ependymomas

Children with ependymomas are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through the Brain Tumor Center, a world-renowned destination for children with malignant and non-malignant brain and spinal cord tumors. Our brain tumor specialists have extensive expertise in treating all types of brain tumors, including ependymomas.

Our areas of research for ependymomas

Clinical trials, or research studies, are ways of evaluating new treatment approaches. Dana-Farber/Boston Children’s is a very experienced and leading institution in developing new treatments for brain tumors. Clinical trials are very important for children with hard-to-treat or relapsed conditions.

It’s possible that your child will be eligible to participate in one of our brain tumor clinical trials. In addition to launching our own clinical trials, we also offer trials available through collaborative groups such as the Children's Oncology Group (COG) and the Pacific Neuro-oncology Consortium (PNOC). We are also the New England Phase I Center of the Children's Oncology Group. If your child has a progressive or recurrent tumor, she may be eligible for a number of clinical trials available through these groups, or from one of our independent clinical investigators.

Ependymoma | Diagnosis & Treatments

How are ependymomas diagnosed?

The first step in treating your child is forming an accurate and complete diagnosis. Ependymomas are most commonly diagnosed from imaging studies and biopsy. Your child's physician will likely order some of the following tests:

There may be other diagnostic tests that your doctor will discuss with you depending on your child's individual situation. After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best possible treatment options.

What are the treatment options for ependymoma?

Your child's physician will determine a specific course of treatment based on several factors, including your child's age, overall health and medical history, the type, location, and size of the tumor and the extent of the disease.

Ependymoma treatments may include neurosurgery, endoscopic third ventriculostomy, radiation or chemotherapy.

Neurosurgery

Surgery is almost always the first step in treating ependymomas. Surgery is usually performed first in order to remove as much of the tumor as possible. Sometimes the tumor's location or other factors may make it impossible for pediatric neurosurgeons to remove it completely. Tumor specimens are examined by neuropathologists to determine the exact diagnosis. Your child's physician will use this information to plan the next phase of treatment.

Endoscopic third ventriculostomy (ETV) or ventriculo-peritoneal shunt (VP shunt)

When a tumor causes blockage of cerebral spinal fluid (CSF) flow, our surgeons may perform one of two procedures in order to relieve symptoms of hydrocephalus, the build up of fluid inside the skull. In an endoscopic third ventriculostomy, neurosurgeons create a small hole that allows fluid to flow around the blockage. About 90 percent of children with symptoms of hydrocephalus will receive this procedure. In some cases, children may have an alternative procedure in which a tube is placed in the ventricles to the abdomen to drain excess fluid into the abdomen. This is called a VP shunt.

Radiation

High-energy waves from a specialized machine damage or shrink tumors. Precisely targeted and dosed radiation therapy is used to kill cancer cells left behind after surgery. Your child may receive this treatment in order to control the local growth of the tumor. If an ependymoma has spread, your child's physician may recommend radiation to the whole brain and spinal cord.

Chemotherapy

Chemotherapy are drugs that interfere with the cancer cells' ability to grow or reproduce, and may be used before surgery, to shrink the tumor. Different groups of chemotherapy drugs work in different ways and are generally systemic treatments. Your child may receive chemotherapy orally, as a pill to swallow; intramuscularly, as an injection into the muscle or fat tissue; intravenously, as a direct injection into the bloodstream, or IV; or intrathecally, as a direct injection into the spinal column through a needle.

Ependymoma treatments can depend on the site of the tumor and whether it is recurrent (doesn't resolve after initial treatment) or not. Within the brain and posterior fossa, outcomes are best when the tumor can be completely removed surgically, and high dose focal radiation therapy is given. For spinal cord ependymomas, a similar approach is used. An ependymoma that has spread, or metastasized, may require additional chemotherapy and radiation depending on the age of your child.

Ependymoma | Programs & Services