The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary.
David N. Louis,Arie Perry,Guido Reifenberger,Andreas von Deimling,Dominique Figarella-Branger,Webster K. Cavenee,Hiroko Ohgaki,Otmar D. Wiestler,Paul Kleihues,David W. Ellison +9 more
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TLDR
The 2016 World Health Organization Classification of Tumors of the Central Nervous System is both a conceptual and practical advance over its 2007 predecessor and is hoped that it will facilitate clinical, experimental and epidemiological studies that will lead to improvements in the lives of patients with brain tumors.Abstract:
The 2016 World Health Organization Classification of Tumors of the Central Nervous System is both a conceptual and practical advance over its 2007 predecessor. For the first time, the WHO classification of CNS tumors uses molecular parameters in addition to histology to define many tumor entities, thus formulating a concept for how CNS tumor diagnoses should be structured in the molecular era. As such, the 2016 CNS WHO presents major restructuring of the diffuse gliomas, medulloblastomas and other embryonal tumors, and incorporates new entities that are defined by both histology and molecular features, including glioblastoma, IDH-wildtype and glioblastoma, IDH-mutant; diffuse midline glioma, H3 K27M-mutant; RELA fusion-positive ependymoma; medulloblastoma, WNT-activated and medulloblastoma, SHH-activated; and embryonal tumour with multilayered rosettes, C19MC-altered. The 2016 edition has added newly recognized neoplasms, and has deleted some entities, variants and patterns that no longer have diagnostic and/or biological relevance. Other notable changes include the addition of brain invasion as a criterion for atypical meningioma and the introduction of a soft tissue-type grading system for the now combined entity of solitary fibrous tumor / hemangiopericytoma-a departure from the manner by which other CNS tumors are graded. Overall, it is hoped that the 2016 CNS WHO will facilitate clinical, experimental and epidemiological studies that will lead to improvements in the lives of patients with brain tumors.read more
Citations
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An Integrated TCGA Pan-Cancer Clinical Data Resource to Drive High-Quality Survival Outcome Analytics.
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New Technologies for Analysis of Extracellular Vesicles
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European Association for Neuro-Oncology (EANO) guideline on the diagnosis and treatment of adult astrocytic and oligodendroglial gliomas
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TL;DR: The European Association for Neuro-Oncology guideline provides recommendations for the clinical care of adult patients with astrocytic and oligodendroglial gliomas, including glioblastomas, based on the 2016 WHO classification of tumours of the central nervous system and on scientific developments since the 2014 guideline.
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References
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C11orf95 – RELA fusions drive oncogenic NF-κB signalling in ependymoma
Matthew Parker,Kumarasamypet M. Mohankumar,Chandanamali Punchihewa,Ricardo Weinlich,James Dalton,Yongjin Li,Ryan P. Lee,Ruth G. Tatevossian,Timothy N. Phoenix,Radhika Thiruvenkatam,Elsie White,Bo Tang,Wilda Orisme,Kirti Gupta,Michael Rusch,Xiang Chen,Yuxin Li,Panduka Nagahawhatte,Erin Hedlund,David Finkelstein,Gang Wu,Sheila A. Shurtleff,John Easton,Kristy Boggs,Donald Yergeau,Bhavin Vadodaria,Heather L. Mulder,Jared Becksford,Pankaj Gupta,Robert Huether,Jing Ma,Guangchun Song,Amar Gajjar,Thomas E. Merchant,Frederick A. Boop,Amy A Smith,Li Ding,Charles Lu,Kerri Ochoa,David Zhao,Robert S. Fulton,Lucinda Fulton,Elaine R. Mardis,Richard K. Wilson,James R. Downing,Douglas R. Green,Jinghui Zhang,David W. Ellison,Richard J. Gilbertson +48 more
TL;DR: The data identify a highly recurrent genetic alteration of RELA in human cancer, and the C11orf95–RELA fusion protein as a potential therapeutic target in supratentorial ependymoma.
Journal ArticleDOI
Medulloblastoma: clinicopathological correlates of SHH, WNT, and non-SHH/WNT molecular subgroups
David W. Ellison,James Dalton,Mehmet Kocak,SL Nicholson,Charles H. Fraga,Geoff Neale,Anna M. Kenney,Daniel J. Brat,Arie Perry,William H. Yong,Roger E. Taylor,Simon Bailey,Steven C. Clifford,Richard J. Gilbertson +13 more
TL;DR: A robust method for detecting SHH, WNT, and non-SHH/WNT molecular subgroups in formalin-fixed medulloblastoma samples is described and the first outcome data based on a clinical trial cohort and novel data on how molecular sub groups are distributed across the range of disease are provided.
Journal ArticleDOI
Whole exome sequencing identifies a recurrent NAB2-STAT6 fusion in solitary fibrous tumors
Juliann Chmielecki,Aimee M. Crago,Mara Rosenberg,Rachael O'Connor,Sarah R. Walker,Sarah R. Walker,Lauren Ambrogio,Daniel Auclair,Aaron McKenna,Michael Heinrich,David A. Frank,David A. Frank,Matthew Meyerson,Matthew Meyerson +13 more
TL;DR: Analysis in 53 tumors confirmed the presence of 7 variants of this fusion transcript in 29 tumors, representing a lower bound for fusion frequency at this locus and suggesting that the NAB2-STAT6 fusion is a distinct molecular feature of SFTs.
Journal ArticleDOI
Interobserver variation of the histopathological diagnosis in clinical trials on glioma: a clinician’s perspective
TL;DR: Although molecular classification may help in typing and grading tumors, as of today this is still in its infancy and unlikely to completely replace histological classification.
Journal ArticleDOI
ATRX and IDH1-R132H immunohistochemistry with subsequent copy number analysis and IDH sequencing as a basis for an "integrated" diagnostic approach for adult astrocytoma, oligodendroglioma and glioblastoma.
David E. Reuss,Felix Sahm,Felix Sahm,Daniel Schrimpf,Daniel Schrimpf,Benedikt Wiestler,Benedikt Wiestler,David Capper,David Capper,Christian Koelsche,Christian Koelsche,Leonille Schweizer,Leonille Schweizer,Andrey Korshunov,Andrey Korshunov,David T.W. Jones,Volker Hovestadt,Michel Mittelbronn,Michel Mittelbronn,Jens Schittenhelm,Christel Herold-Mende,Andreas Unterberg,Michael Platten,Michael Platten,Michael Weller,Wolfgang Wick,Wolfgang Wick,Stefan M. Pfister,Stefan M. Pfister,Andreas von Deimling,Andreas von Deimling +30 more
TL;DR: An algorithm based on stepwise analysis with initial immunohistochemistry for ATRX and IDH1-R132H followed by 1p/19q analysis followed by IDH sequencing which reduces the number of molecular analyses and which has a far better association with patient outcome than WHO 2007 is presented.
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