Ependymoma

Ependymoma is a rare tumor of the central nervous system (CNS) that arises from ependymal cells, which line the ventricles of the brain and the central canal of the spinal cord. These tumors can occur at any age but are particularly common in young children and represent approximately 5-10% of all childhood CNS tumors. Ependymomas are classified by the World Health Organization (WHO) into different grades, including subependymoma (grade I), classic ependymoma (grade II), and anaplastic ependymoma (grade III). The most common locations include the posterior fossa (infratentorial region) in children and the spinal cord in adults. Symptoms depend on the tumor's location and size. Intracranial ependymomas may cause headaches, nausea, vomiting, balance difficulties, visual disturbances, and signs of increased intracranial pressure such as hydrocephalus. Spinal ependymomas can present with back pain, weakness, numbness, or bowel and bladder dysfunction. In infants, symptoms may include irritability, failure to thrive, and increasing head circumference. The primary treatment for ependymoma is maximal safe surgical resection, which is the most important prognostic factor. Radiation therapy is commonly used as adjuvant treatment, particularly when complete surgical removal is not achievable or in higher-grade tumors. The role of chemotherapy remains limited and is primarily used in very young children to delay radiation therapy or in recurrent disease. Molecular classification of ependymomas, including subtypes such as RELA fusion-positive supratentorial ependymoma and posterior fossa group A (PFA) ependymoma, is increasingly guiding prognosis and treatment strategies. Despite advances, recurrence remains a significant challenge, and long-term follow-up with serial imaging is essential.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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