Central nervous system ependymoma: clinical implications of the new molecular classification, treatment guidelines and controversial issues
作者： P. D. Delgado-López , E. M. Corrales-García , E. Alonso-García , R. García-Leal , R. González-Rodrigálvarez
DOI: 10.1007/S12094-019-02082-2
关键词:
摘要: Ependymoma is an uncommon neuroepithelial tumor that may arise anywhere within the neuroaxis, both in children and adults. It has been classically graded upon histopathological features, yet with limited clinical utility. Recently, DNA methylation profiling provided a novel classification of ependymoma nine molecular subgroups. This stratification method harbors prognostic value supratentorial RELA-fusion posterior fossa group A tumors showing significantly shorter survival compared to rest. Currently, treatment choice involves maximal safe resection and, cases residual disease, adjuvant conformal radiotherapy. Second-look surgery also feasible recommended option for incompletely resected tumors. The role chemotherapy not established can be considered infants relapsing disease or prior re-intervention. Although targeted agents do seem play as therapy, they are currently being tested recurrent disease.
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