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

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

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.

Keywords: Ependymoma; Posterior fossa; RELA; Subgroups; Treatment; Trial; YAP1.

Figures

Fig. 1
Fig. 1
General and molecular subgroup specific consensus statements on the clinical management of intracranial ependymoma

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