Anaplastic ependymoma

Anaplastic ependymoma (also known as WHO grade III ependymoma or malignant ependymoma) is a rare, aggressive brain tumor that arises from ependymal cells, which line the ventricles of the brain and the central canal of the spinal cord. It is classified as a high-grade (grade III) variant of ependymoma, distinguished from lower-grade ependymomas by features of increased cellularity, frequent mitotic activity, microvascular proliferation, and necrosis on histopathological examination. This tumor primarily affects the central nervous system and can occur in both the brain (intracranially) and, less commonly, the spinal cord. Symptoms depend on the tumor's location but commonly include headaches, nausea, vomiting, seizures, and neurological deficits such as weakness, balance difficulties, or visual disturbances. In children, tumors frequently arise in the posterior fossa (near the brainstem and cerebellum), which can lead to hydrocephalus due to obstruction of cerebrospinal fluid flow, causing increased intracranial pressure. In adults, supratentorial locations are more common. Anaplastic ependymomas can occur at any age but are more frequently diagnosed in children and young adults. The current treatment approach typically involves maximal safe surgical resection as the primary intervention, followed by adjuvant radiation therapy. The extent of surgical resection is one of the most important prognostic factors. The role of chemotherapy remains less well-defined, though it may be considered in recurrent disease or in very young children to delay radiation therapy. Despite treatment, anaplastic ependymomas carry a higher risk of recurrence and a poorer prognosis compared to lower-grade ependymomas. Ongoing research is exploring molecular subtypes of ependymoma to better guide targeted therapeutic strategies.

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

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