The current consensus on the clinical management of intracranial ependymoma and its distinct molecular variants

Kristian W Pajtler,Amy Smith,Richard G Grundy,Christian A Smith,Cécile Faure‐Conter ,Eui-Hyoung Hwang ,David W Ellison,Hendrik Witt,Stephen C Mack,Yonehiro Kanemura,Andrey Korshunov,Richard J Gilbertson,Thomas E Merchant,David Castel,Piergiorgio Modena,Kenneth Aldape ,Uri Tabori,Nicholas K Foreman,Amar Gajjar,Peter B Dirks,Éric Bouffet ,Stefan M Pfister,Koichi Ichimura,Vijay Ramaswamy,Katherine E Warren,Andreas Von Deimling,Jonathan L Finlay ,Felice Giangaspero,Eric C Holland,Didier Frappaz,Katja Von Hoff,Maura Massimino,Karen Wright ,Marcel Kool,Jordan R Hansford,Michael D Taylor

doi:10.1007/s00401-016-1643-0

Abstract

Multiple independent genomic profiling efforts have recently identified clinically and molecularly distinct subgroups of ependymoma arising from all three anatomic compartments of the central nervous system (supratentorial brain, posterior fossa, and spinal cord). These advances motivated a consensus meeting to discuss: (1) the utility of current histologic grading criteria, (2) the integration of molecular-based stratification schemes in future clinical trials for patients with ependymoma and (3) current therapy in the context of molecular subgroups. Discussion at the meeting generated a series of consensus statements and recommendations from the attendees, which comment on the prognostic evaluation and treatment decisions of patients with intracranial ependymoma (WHO Grade II/III) based on the knowledge of its molecular subgroups. The major consensus among attendees was reached that treatment decisions for ependymoma (outside of clinical trials) should not be based on grading (II vs III). Supratentorial and posterior fossa ependymomas are distinct diseases, although the impact on therapy is still evolving. Molecular subgrouping should be part of all clinical trials henceforth.

Highlights

Ependymoma is a histologically defined intrinsic tumor that involves the three major anatomic compartments of the central nervous system and affects both children and adults
Discussion at the meeting generated a series of consensus statements and recommendations from the attendees, which comment on the prognostic evaluation and treatment decisions of patients with intracranial ependymoma (WHO Grade II/III) based on the knowledge of its molecular subgroups
Recent advances in the biological characterization of ependymal tumors have demonstrated the existence of nine clinically, demographically, and molecularly distinct entities, with three occurring in each anatomic compartment. These findings offer new opportunities to create a precise, reliable, and objective platform for stratification of ependymoma patients, and the potential for altering therapeutic decisions based on molecular features

Summary

Introduction

Ependymoma is a histologically defined intrinsic tumor that involves the three major anatomic compartments (supratentorial brain, posterior fossa, and spinal cord) of the central nervous system and affects both children and adults. Discussion at the meeting generated a series of consensus statements and recommendations from the attendees, which comment on the prognostic evaluation and treatment decisions of patients with intracranial ependymoma (WHO Grade II/III) based on the knowledge of its molecular subgroups.

Results

Conclusion