title: The current consensus on the clinical management of intracranial ependymoma and its distinct molecular variants.
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.
authors.affiliation: Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ), Heidelberg, Germany.|Department of Pediatric Oncology, Hematology and Immunology, University Hospital Heidelberg, Heidelberg, Germany.|German Cancer Consortium (DKTK), Heidelberg, Germany.
authors.forename: Kristian W
authors.initials: KW
authors.lastname: Pajtler
authors.affiliation: Department of Stem Cell Biology and Regenerative Medicine, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, USA.|Department of Molecular Medicine, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Stephen C
authors.initials: SC
authors.lastname: Mack
authors.affiliation: Division of Neurosurgery, Arthur & Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, Toronto, ON, Canada.|Division of Hematology/Oncology, Hospital for Sick Children, Toronto, ON, Canada.
authors.forename: Vijay
authors.initials: V
authors.lastname: Ramaswamy
authors.affiliation: Division of Neurosurgery, Arthur & Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, Toronto, ON, Canada.
authors.forename: Christian A
authors.initials: CA
authors.lastname: Smith
authors.affiliation: Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ), Heidelberg, Germany.|Department of Pediatric Oncology, Hematology and Immunology, University Hospital Heidelberg, Heidelberg, Germany.|German Cancer Consortium (DKTK), Heidelberg, Germany.
authors.forename: Hendrik
authors.initials: H
authors.lastname: Witt
authors.affiliation: Arnold Palmer Hospital, Orlando, FL, USA.
authors.forename: Amy
authors.initials: A
authors.lastname: Smith
authors.affiliation: Royal Children's Hospital, Melbourne, VIC, Australia.
authors.forename: Jordan R
authors.initials: JR
authors.lastname: Hansford
authors.affiliation: Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors.forename: Katja
authors.initials: K
authors.lastname: von Hoff
authors.affiliation: Department of Oncology, St Jude Children's Research Hospital, Memphis, TN, USA.
authors.forename: Karen D
authors.initials: KD
authors.lastname: Wright
authors.affiliation: Center for Cancer and Blood Disorders, Children's National Medical Center, Washington, DC, USA.
authors.forename: Eugene
authors.initials: E
authors.lastname: Hwang
authors.affiliation: Pediatric Neuro-Oncology Centre Léon Bérard, Lyon, France.
authors.forename: Didier
authors.initials: D
authors.lastname: Frappaz
authors.affiliation: Department of Neurosurgery and Institute for Clinical Research, Osaka National Hospital, Osaka, Japan.
authors.forename: Yonehiro
authors.initials: Y
authors.lastname: Kanemura
authors.affiliation: Fondazione IRCCS-Istituto Nazionale dei Tumori, Milan, Italy.
authors.forename: Maura
authors.initials: M
authors.lastname: Massimino
authors.affiliation: Pediatric Neuro-Oncology Centre Léon Bérard, Lyon, France.
authors.forename: Cécile
authors.initials: C
authors.lastname: Faure-Conter
authors.affiliation: Laboratory of Genetics, Pathology Unit, S. Anna General Hospital, Como, Italy.
authors.forename: Piergiorgio
authors.initials: P
authors.lastname: Modena
authors.affiliation: Division of Hematology/Oncology, Hospital for Sick Children, Toronto, ON, Canada.
authors.forename: Uri
authors.initials: U
authors.lastname: Tabori
authors.affiliation: National Cancer Institute, National Institute of Health, Bethesda, MD, USA.
authors.forename: Katherine E
authors.initials: KE
authors.lastname: Warren
authors.affiliation: Division of Human Biology, Fred Hutchinson Cancer Research Center, Seattle, WA, USA.
authors.forename: Eric C
authors.initials: EC
authors.lastname: Holland
authors.affiliation: Division of Brain Tumor Translational Research, National Cancer Center Research Institute, Tokyo, Japan.
authors.forename: Koichi
authors.initials: K
authors.lastname: Ichimura
authors.affiliation: Department of Radiological Sciences, Oncology and Anatomical Pathology, Sapienza University, Rome, Italy.
authors.forename: Felice
authors.initials: F
authors.lastname: Giangaspero
authors.affiliation: Département de Cancérologie de l'Enfant et de l'Adolescent, Gustave Roussy, Univ. Paris-Sud, Université Paris-Saclay, Villejuif, France.|UMR8203 "Vectorologie and Thérapeutiques Anticancéreuses", CNRS, Gustave Roussy, Univ. Paris-Sud, Université Paris-Saclay, Villejuif, France.
authors.forename: David
authors.initials: D
authors.lastname: Castel
authors.affiliation: Department of Neuropathology, University of Heidelberg, Heidelberg, Germany.|Clinical Cooperation Unit Neuropathology, German Cancer Research Center (DKFZ), Heidelberg, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: von Deimling
authors.affiliation: Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ), Heidelberg, Germany.|German Cancer Consortium (DKTK), Heidelberg, Germany.
authors.forename: Marcel
authors.initials: M
authors.lastname: Kool
authors.affiliation: Division of Neurosurgery, Arthur & Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, Toronto, ON, Canada.
authors.forename: Peter B
authors.initials: PB
authors.lastname: Dirks
authors.affiliation: Children's Brain Tumour Research Centre, The Medical School, University of Nottingham, Nottingham, UK.
authors.forename: Richard G
authors.initials: RG
authors.lastname: Grundy
authors.affiliation: Department of Pediatrics, University of Colorado Denver, Aurora, CO, USA.
authors.forename: Nicholas K
authors.initials: NK
authors.lastname: Foreman
authors.affiliation: Department of Oncology, St Jude Children's Research Hospital, Memphis, TN, USA.
authors.forename: Amar
authors.initials: A
authors.lastname: Gajjar
authors.affiliation: Department of Neuropathology, University of Heidelberg, Heidelberg, Germany.|Clinical Cooperation Unit Neuropathology, German Cancer Research Center (DKFZ), Heidelberg, Germany.
authors.forename: Andrey
authors.initials: A
authors.lastname: Korshunov
authors.affiliation: Nationwide Children's Hospital and the Ohio State University, Columbus, OH, USA.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Finlay
authors.affiliation: Li Ka Shing Centre, CRUK Cambridge Institute, University of Cambridge, Cambridge, UK.
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Gilbertson
authors.affiliation: Department of Pathology, St Jude Children's Research Hospital, Memphis, TN, USA.
authors.forename: David W
authors.initials: DW
authors.lastname: Ellison
authors.affiliation: Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada.
authors.forename: Kenneth D
authors.initials: KD
authors.lastname: Aldape
authors.affiliation: Department of Radiological Sciences, St Jude Children's Research Hospital, Memphis, TN, USA.
authors.forename: Thomas E
authors.initials: TE
authors.lastname: Merchant
authors.affiliation: Division of Hematology/Oncology, Hospital for Sick Children, Toronto, ON, Canada.
authors.forename: Eric
authors.initials: E
authors.lastname: Bouffet
authors.affiliation: Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ), Heidelberg, Germany. s.pfister@dkfz.de.|Department of Pediatric Oncology, Hematology and Immunology, University Hospital Heidelberg, Heidelberg, Germany. s.pfister@dkfz.de.|German Cancer Consortium (DKTK), Heidelberg, Germany. s.pfister@dkfz.de.
authors.forename: Stefan M
authors.initials: SM
authors.lastname: Pfister
authors.affiliation: Division of Neurosurgery, Arthur & Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, Toronto, ON, Canada. mdtaylor@sickkids.ca.
authors.forename: Michael D
authors.initials: MD
authors.lastname: Taylor
mesh_terms: D000818:Animals; D001932:Brain Neoplasms / Q000235:genetics / Q000378:metabolism* / Q000473:pathology / Q000628:therapy*; D032921:Consensus; D019468:Disease Management; D004806:Ependymoma / Q000235:genetics / Q000378:metabolism* / Q000473:pathology / Q000628:therapy*; D006801:Humans; D009367:Neoplasm Staging
keywords: Ependymoma; Posterior fossa; RELA; Subgroups; Treatment; Trial; YAP1