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

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 i...

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Autores: Gielen GH, Baugh JN, van Vuurden DG, Veldhuijzen van Zanten SEM, Hargrave D, Massimino M, Biassoni V, Morales La Madrid A, Karremann M, Wiese M, Thomale U, Janssens GO, von Bueren AO, Perwein T, Nussbaumer G, Hoving EW, Niehusmann P, Gessi M, Kwiecien R, Bailey S, Pietsch T, Andreiuolo F, Kramm CM
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2022
País:España
Institución:Fundació Sant Joan de Déu
Repositorio:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
OAI Identifier:oai:fsjd.fundanetsuite.com:p21768
Acceso en línea:https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=21768
Access Level:acceso abierto
Palabra clave:diffuse midline glioma
DIPG
pediatric high-grade glioma
World Health Organization
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spelling Pediatric high-grade gliomas and the WHO CNS Tumor Classification-Perspectives of pediatric neuro-oncologists and neuropathologists in light of recent updatesGielen GHBaugh JNvan Vuurden DGVeldhuijzen van Zanten SEMHargrave DMassimino MBiassoni VMorales La Madrid AKarremann MWiese MThomale UJanssens GOvon Bueren AOPerwein TNussbaumer GHoving EWNiehusmann PGessi MKwiecien RBailey SPietsch TAndreiuolo FKramm CMdiffuse midline gliomaDIPGpediatric high-grade gliomaWorld Health OrganizationBackground 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.OXFORD UNIV PRESS2022info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttps://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=21768Neuro-Oncology AdvancesISSN: 26322498reponame:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déuinstname:Fundació Sant Joan de DéuInglésinfo:eu-repo/semantics/openAccessoai:fsjd.fundanetsuite.com:p217682026-05-27T12:37:41Z
dc.title.none.fl_str_mv Pediatric high-grade gliomas and the WHO CNS Tumor Classification-Perspectives of pediatric neuro-oncologists and neuropathologists in light of recent updates
title Pediatric high-grade gliomas and the WHO CNS Tumor Classification-Perspectives of pediatric neuro-oncologists and neuropathologists in light of recent updates
spellingShingle Pediatric high-grade gliomas and the WHO CNS Tumor Classification-Perspectives of pediatric neuro-oncologists and neuropathologists in light of recent updates
Gielen GH
diffuse midline glioma
DIPG
pediatric high-grade glioma
World Health Organization
title_short Pediatric high-grade gliomas and the WHO CNS Tumor Classification-Perspectives of pediatric neuro-oncologists and neuropathologists in light of recent updates
title_full Pediatric high-grade gliomas and the WHO CNS Tumor Classification-Perspectives of pediatric neuro-oncologists and neuropathologists in light of recent updates
title_fullStr Pediatric high-grade gliomas and the WHO CNS Tumor Classification-Perspectives of pediatric neuro-oncologists and neuropathologists in light of recent updates
title_full_unstemmed Pediatric high-grade gliomas and the WHO CNS Tumor Classification-Perspectives of pediatric neuro-oncologists and neuropathologists in light of recent updates
title_sort Pediatric high-grade gliomas and the WHO CNS Tumor Classification-Perspectives of pediatric neuro-oncologists and neuropathologists in light of recent updates
dc.creator.none.fl_str_mv Gielen GH
Baugh JN
van Vuurden DG
Veldhuijzen van Zanten SEM
Hargrave D
Massimino M
Biassoni V
Morales La Madrid A
Karremann M
Wiese M
Thomale U
Janssens GO
von Bueren AO
Perwein T
Nussbaumer G
Hoving EW
Niehusmann P
Gessi M
Kwiecien R
Bailey S
Pietsch T
Andreiuolo F
Kramm CM
author Gielen GH
author_facet Gielen GH
Baugh JN
van Vuurden DG
Veldhuijzen van Zanten SEM
Hargrave D
Massimino M
Biassoni V
Morales La Madrid A
Karremann M
Wiese M
Thomale U
Janssens GO
von Bueren AO
Perwein T
Nussbaumer G
Hoving EW
Niehusmann P
Gessi M
Kwiecien R
Bailey S
Pietsch T
Andreiuolo F
Kramm CM
author_role author
author2 Baugh JN
van Vuurden DG
Veldhuijzen van Zanten SEM
Hargrave D
Massimino M
Biassoni V
Morales La Madrid A
Karremann M
Wiese M
Thomale U
Janssens GO
von Bueren AO
Perwein T
Nussbaumer G
Hoving EW
Niehusmann P
Gessi M
Kwiecien R
Bailey S
Pietsch T
Andreiuolo F
Kramm CM
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv diffuse midline glioma
DIPG
pediatric high-grade glioma
World Health Organization
topic diffuse midline glioma
DIPG
pediatric high-grade glioma
World Health Organization
description 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.
publishDate 2022
dc.date.none.fl_str_mv 2022
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=21768
url https://fsjd.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=21768
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv OXFORD UNIV PRESS
publisher.none.fl_str_mv OXFORD UNIV PRESS
dc.source.none.fl_str_mv Neuro-Oncology Advances
ISSN: 26322498
reponame:r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
instname:Fundació Sant Joan de Déu
instname_str Fundació Sant Joan de Déu
reponame_str r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
collection r-FSJD. Repositorio Institucional de Producción Científica de la Fundació Sant Joan de Déu
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