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Ependymomas arise from cells lining the passageways in the brain that produce and store the cerebrospinal fluid or CSF. (CSF is a fluid that bathes the brain and spinal cord.) These tumors are classified as either supratentorial (in the top part of the head) or infratentorial (in the back of the head). In children, the majority of ependymomas occur as infratentorial tumors arising in or around the fluid-filled fourth ventricle.
Ependymomas represent approximately six percent of brain tumors in children. The majority of patients are diagnosed before the age of five years.
The location of the tumor determines the clinical features and symptoms. Patients present with the common symptoms of increased pressure on the brain--nausea, vomiting, and headache. Patients with infratentorial tumors may also have unsteady gait, problems with coordination, and neck pain or stiffness resulting from irritation of the upper cervical nerves by the tumor. Supratentorial tumors usually present with complaints of headaches, weakness in the arms and legs, and sometimes, visual abnormalities (blurred vision). Very few ependymomas spread to other parts of the brain or to the spinal cord before they are diagnosed.
Surgery and radiation therapy are the main treatment approaches. Aggressive surgical resection is strongly recommended prior to radiation therapy in order to ensure the best possible long term outcome.
The dose and the "field" of radiation is dependent on the location of the tumor and the specific features of the tumor cells (histology). Chemotherapy generally has not proved beneficial in previous clinical studies, but may be benecial in a limited setting prior to more surgery or if there is tumor recurrence.
If there is residual tumor, newer chemotherapeutic agents are being used after surgery, with the goal of shrinking the tumor before planned radiation therapy. Radiation oncologists are now using focal radiation techniques after surgery with excellent long term results. Another approach that is being studied is to consider repeated surgery in patients who still have tumor remaining after surgery and radiation therapy. The best survival rates are obtained in patients who have no visible tumor after surgery.
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