Central nervous system ependymoma: clinical implications of the new molecular classification, treatment guidelines and controversial issues

Abstract

Ependymoma is an uncommon neuroepithelial tumor that may arise anywhere within the neuroaxis, both in children and in adults. It has been classically graded upon histopathological features, yet with limited clinical utility. Recently, DNA methylation profiling has provided a novel classification of ependymoma in nine molecular subgroups. This stratification method harbors prognostic value with supratentorial RELA-fusion and posterior fossa group A tumors showing a significantly shorter survival compared to the rest. Currently, the treatment of choice involves maximal safe resection and, in cases of residual disease, adjuvant conformal radiotherapy. Second-look surgery is also a feasible and recommended option for incompletely resected tumors. The role of chemotherapy is not yet established and can be considered in infants and children with relapsing disease or prior to re-intervention. Although targeted agents do not seem to play a role as adjuvant therapy, they are currently being tested for recurrent disease.

Keywords: Chemotherapy; DNA profile; Ependymoma; Prognosis; Radiotherapy; Resection.