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The clinical and molecular landscape of diffuse hemispheric glioma, H3 G34-mutant

Diffuse hemispheric glioma, histone 3 (H3) G34-mutant, has been newly defined in the 2021 World Health Organization (WHO) classification of central nervous system tumors. Here we sought to define the prognostic roles of clinical, neuroimaging, pathological, and molecular features of these tumors.We...

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Main Authors: Le Rhun, Emilie (Author) , Bink, Andrea (Author) , Felsberg, Joerg (Author) , Gramatzki, Dorothee (Author) , Brandner, Sebastian (Author) , Benhamida, Jamal K (Author) , Wick, Antje (Author) , Tonn, Joerg C (Author) , Mohme, Malte (Author) , Tabatabai, Ghazaleh (Author) , Capper, David (Author) , Snuderl, Matija (Author) , Razis, Evangelia (Author) , Ronellenfitsch, Michael Wilfried (Author) , Neidert, Nicolas (Author) , Ng, Ho-Keung (Author) , Pohl, Ute (Author) , Bale, Tejus (Author) , Quach, Stefanie (Author) , Rieger, David (Author) , Schüller, Ulrich (Author) , Onken, Julia (Author) , Drüschler, Katharina (Author) , Maurage, Claude-Alain (Author) , Regli, Luca (Author) , Healy, Estelle (Author) , Graham, Maya (Author) , Hortobagyi, Tibor (Author) , Paine, Simon (Author) , Bridges, Leslie (Author) , Lausová, Tereza (Author) , Medici, Valentina (Author) , Sievers, Philipp (Author) , Schrimpf, David (Author) , Wick, Wolfgang (Author) , Sahm, Felix (Author) , Reifenberger, Guido (Author) , Deimling, Andreas von (Author) , Weller, Michael (Author)
Format: Article (Journal)
Language:English
Published: June 2025
In: Neuro-Oncology
Year: 2025, Volume: 27, Issue: 6, Pages: 1519-1535
ISSN:1523-5866
DOI:10.1093/neuonc/noaf015
Online Access:Verlag, kostenfrei, Volltext: https://doi.org/10.1093/neuonc/noaf015
Verlag, kostenfrei, Volltext: https://academic.oup.com/neuro-oncology/article/27/6/1519/7972761?login=true
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Author Notes:Emilie Le Rhun, Andrea Bink, Joerg Felsberg, Dorothee Gramatzki, Sebastian Brandner, Jamal K. Benhamida, Antje Wick, Joerg C. Tonn, Malte Mohme, Ghazaleh Tabatabai, David Capper, Matija Snuderl, Evangelia Razis, Michael W. Ronellenfitsch, Nicolas Neidert, Ho-Keung Ng, Ute Pohl, Tejus Bale, Stefanie Quach, David Rieger, Ulrich Schüller, Julia Onken, Katharina Drüschler, Claude-Alain Maurage, Luca Regli, Estelle Healy, Maya Graham, Tibor Hortobagyi, Simon Paine, Leslie Bridges, Tereza Lausova, Valentina Medici, Philipp Sievers, David Schrimpf, Wolfgang Wick, Felix Sahm, Guido Reifenberger, Andreas von Deimling, Michael Weller, for the H3 G34 DHG Study Group
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Summary:Diffuse hemispheric glioma, histone 3 (H3) G34-mutant, has been newly defined in the 2021 World Health Organization (WHO) classification of central nervous system tumors. Here we sought to define the prognostic roles of clinical, neuroimaging, pathological, and molecular features of these tumors.We retrospectively assembled a cohort of 114 patients (median age 22 years) with diffuse hemispheric glioma, H3 G34-mutant, central nervous system WHO grade 4, and profiled the imaging, histological, and molecular landscape of their tumors.Compared with glioblastoma, H3 G34-mutant diffuse hemispheric gliomas exhibited less avid contrast enhancement, necrosis, and edema on MRI. Comprehensive analyses of mutational and DNA copy number profiles revealed recurrent mutations in TP53 and ATRX, homozygous deletions of CDKN2A/B, and amplifications of PDGFRA, EGFR, CCND2, and MYCN. MGMT promoter methylation was detected in 79 tumors (75%); 11 tumors (13%) showed DNA copy number profiles suggestive of circumscribed deletions on 10q26.3 involving the MGMT locus. Median survival was 21.5 months. Female sex, gross total resection, and MGMT promoter methylation were positive prognostic factors on univariate analysis. Among radiological, pathological, and molecular features, the absence of pial invasion and the presence of microvascular proliferation and CDK6 amplification were positive prognostic factors on univariate analyses.This study refines the clinical and molecular landscape of H3 G34-mutant diffuse hemispheric gliomas. Dedicated trials for this novel tumor type are urgently needed.
Item Description:Online verfügbar: 22. Januar 2025, Artikelversion: 06. Mai 2025
Gesehen am 17.10.2025
Physical Description:Online Resource
ISSN:1523-5866
DOI:10.1093/neuonc/noaf015

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