Ependymomas

Ependymal tumors are glial neoplasms believed to arise from ependymal cells that line the ventricular system. Ependymomas may occur anywhere along the neuroaxis and Grade 1 tumors (myxopapillary ependymomas and subependymomas) are biologically distinct from Grade 2 (low grade) and Grade 3 (anaplastic) ependymomas. The molecular pathology of ependymomas is poorly understood. Although a consensus regarding management of ependymomas is lacking, a basic tenet of treatment, for both pediatric and adult tumors, is the need for a gross total resection, if feasible. The role of radiation therapy is not as clear, but most would recommend focal radiation therapy for patients with subtotally resected tumors, for anaplastic tumors, and possibly for those with totally resected (especially posterior fossa) tumors. The benefit of chemotherapy, as regards long-term tumor control, is unclear.