Ependymal tumor

Ependymal tumors (also known as ependymomas) are a group of rare central nervous system (CNS) neoplasms that arise from ependymal cells, which line the ventricles of the brain and the central canal of the spinal cord. These tumors belong to the broader category of glial tumors and can occur at any level of the neuraxis, including the posterior fossa (most common in children), the supratentorial compartment, and the spinal cord (more common in adults). Subtypes include subependymoma, myxopapillary ependymoma, classic ependymoma, and anaplastic ependymoma, each with distinct clinical behavior and prognosis. Key symptoms depend on tumor location and may include headaches, nausea, vomiting, balance difficulties, and visual disturbances when the tumor is intracranial, often due to obstructive hydrocephalus. Spinal ependymomas may present with back pain, weakness, numbness, or bowel and bladder dysfunction. In young children, irritability, developmental regression, and increasing head circumference may be early signs. The current treatment landscape centers on maximal safe surgical resection, which is the most important prognostic factor. Adjuvant radiation therapy is frequently employed, particularly for incompletely resected tumors or higher-grade lesions. The role of chemotherapy remains limited and is primarily investigated in clinical trials, especially for recurrent disease or in very young children where radiation is deferred. Recent molecular classification has identified distinct subgroups (such as RELA-fusion positive supratentorial ependymoma and posterior fossa group A) that carry different prognoses and may guide future targeted therapies. Long-term follow-up is essential due to the risk of local recurrence.

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

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