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Pediatric high-grade gliomas and the WHO CNS Tumor Classification - Perspectives of pediatric neuro-oncologists and neuropathologists in light of recent updates

Gielen, GH; Baugh, JN; Van Vuurden, DG; Veldhuijzen Van Zanten, SEM; Hargrave, D; Massimino, M; Biassoni, V; ... Kramm, CM; + view all (2022) Pediatric high-grade gliomas and the WHO CNS Tumor Classification - Perspectives of pediatric neuro-oncologists and neuropathologists in light of recent updates. Neuro-Oncology Advances , 4 (1) , Article vdac077. 10.1093/noajnl/vdac077. Green open access

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Abstract

Background: The WHO Classification of Tumors of the Central Nervous System has undergone major restructuring. Molecularly defined diagnostic criteria were introduced in 2016 (revised 4th edition) and expanded in 2021 (5th edition) to incorporate further essential diagnostic molecular parameters. We investigated potential differences between specialists in perception of these molecularly defined subtypes for pediatric high-grade gliomas (pedHGG). Methods: We designed a 22-question survey studying the impact of the revised 4th edition of the WHO classification on pedHGG. Data were collected and statistically analyzed to examine the spectrum of viewpoints and possible differences between neuro-oncologists and neuropathologists. Results: 465 participants from 53 countries were included; 187 pediatric neuro-oncologists (40%), 160 neuropathologists (34%), and 118 additional experts (26%). Neuro-oncologists reported issues with the introduction of molecularly defined tumor types, as well as the abolishment or renaming of established tumor entities, while neuropathologists did not to the same extent. Both groups indicated less relevant or insufficient diagnostic definitions were available in 2016. Reported issues were classified and assessed in the 2021 WHO classification and a substantial improvement was perceived. However, issues of high clinical relevance remain to be addressed, including the definition of clinical phenotypes for diffuse intrinsic pontine glioma and gliomatosis cerebri. Conclusions: Within the WHO classification of pediatric brain tumors, such as pedHGG, rapid changes in molecular characterization have been introduced. This study highlights the ongoing need for cross talk between pathologist and oncologist to advance the classification of pedHGG subtypes and ensure biological relevance and clinical impact.

Type: Article
Title: Pediatric high-grade gliomas and the WHO CNS Tumor Classification - Perspectives of pediatric neuro-oncologists and neuropathologists in light of recent updates
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1093/noajnl/vdac077
Publisher version: https://doi.org/10.1093/noajnl/vdac077
Language: English
Additional information: This work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third-party material in this article are included in the Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/
Keywords: DIPG, World Health Organization, diffuse midline glioma, pediatric high-grade glioma
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Biology and Cancer Dept
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
URI: https://discovery.ucl.ac.uk/id/eprint/10153966
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