TY - JOUR
T1 - Pediatric high-grade gliomas and the WHO CNS Tumor Classification-Perspectives of pediatric neuro-oncologists and neuropathologists in light of recent updates
AU - Gielen, Gerrit H
AU - Baugh, Joshua N
AU - van Vuurden, Dannis G
AU - Veldhuijzen van Zanten, Sophie E M
AU - Hargrave, Darren
AU - Massimino, Maura
AU - Biassoni, Veronica
AU - Morales la Madrid, Andres
AU - Karremann, Michael
AU - Wiese, Maria
AU - Thomale, Ulrich
AU - Janssens, Geert O
AU - von Bueren, André O
AU - Perwein, Thomas
AU - Nussbaumer, Gunther
AU - Hoving, Eelco W
AU - Niehusmann, Pitt
AU - Gessi, Marco
AU - Kwiecien, Robert
AU - Bailey, Simon
AU - Pietsch, Torsten
AU - Andreiuolo, Felipe
AU - Kramm, Christof M
N1 - Publisher Copyright:
© 2022 The Author(s). Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.
PY - 2022/1/1
Y1 - 2022/1/1
N2 - 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.
AB - 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.
KW - diffuse midline glioma
KW - DIPG
KW - pediatric high-grade glioma
KW - World Health Organization
UR - http://www.scopus.com/inward/record.url?scp=85134924853&partnerID=8YFLogxK
U2 - 10.1093/noajnl/vdac077
DO - 10.1093/noajnl/vdac077
M3 - Article
C2 - 35733513
SN - 2632-2498
VL - 4
JO - Neuro-oncology advances
JF - Neuro-oncology advances
IS - 1
M1 - vdac077
ER -