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Ependymoma


 

Definition: Ependymomas are tumors that arise from ependymal cells that line the venticles in the brain and the center of the spinal cord where cerebrospinal fluid flows. The World Health Organization classifies ependymomas as grade I (myxopapillary ependymoma and subependymoma), grade II (ependymoma), or grade III (anaplastic ependymoma) based on the aggressiveness of the tumor. Ependymomas can occur in the brain and spread to the spinal cord through the cerebrospinal fluid. The majority of ependymomas are benign (grade I).

Symptoms: Many ependymomas occur in the fourth ventricle and can block the flow of cerebrospinal fluid leading to hydrocephalus, a build-up of cerebrospinal fluid in the brain.  Increased intracranial pressure from hydrocephalus can cause headaches, nausea, vomiting, visual changes, and changes in behavior. Ependymomas in the spine can cause back pain, weakness in the arms or legs, or bladder problems.

Evaluation: Ependymomas are evaluated with an MRI or CT scan. A lumbar puncture (spinal tap) may be performed to see if there are ependymoma cells in the cerebrospinal fluid.

Treatment Options:

  • Surgery: Surgical biopsy and removal of as much of the tumor as possible without causing significant neurological damage is the first step in the treatment of an ependymoma.
  • Radiation: Ependymomas are very sensitive to radiation so fractionated radiation is usually recommended after surgery to prevent tumor re-growth.
  • Chemotherapy: Chemotherapy is occasionally used in the treatment of ependymomas. 

Follow-up: Long-term follow-up with regular scans and neurological exams is recommended to watch for tumor recurrence.