Ependymomas

Ependymomas are a heterogeneous group of primary central nervous system neoplasms with different clinico-radiological characteristics, pathological and molecular features, and survival outcomes. Surgical resection represents the gold standard with gross total resection feasible in up to 60–70% of patients. Adjuvant radiotherapy is standard of care in grade III anaplastic and in incompletely resected grade II tumors. Reoperation and reirradiation (including stereotactic radiotherapy) are increasingly adopted at recurrence. Chemotherapy can be useful as salvage therapy, using temozolomide or platinum compounds. Myxopapillary ependymomas are not considered benign tumors any more, as they tend to recur and/or disseminate in the cerebrospinal fluid. New molecular alterations are being discovered in ependymomas, and this will allow them in the future to be subdivided into molecularly based subgroups that can be targeted with specific compounds.