biostudies-literatureLe Rhun EGerman Research FoundationSwiss National Science Foundationthe German Cancer AidNCI NIH HHSthe Koetser Foundation1519-1535https://www.ebi.ac.uk/biostudies/studies/S-EPMC12309718biostudies-literatureUnknown27(6)<h4>Background</h4>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.<h4>Methods</h4>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.<h4>Results</h4>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 revealNeuro-oncologyThe clinical and molecular landscape of diffuse hemispheric glioma, H3 G34-mutant.PMC12309718P30 CA008748Grant 70-3163-Wi 3185155310030Grant 310030_185155Weller MPohl URieger DOnken JBrandner SBridges LSievers PWick ANg HKSchrimpf DSnuderl MPaine SBenhamida JKTabatabai GLausova TGramatzki DBink AReifenberger GRonellenfitsch MWHortobagyi TMaurage CABale Tvon Deimling ATonn JCNeidert NRegli LCapper DGraham MQuach SMedici VSahm FHealy ELe Rhun EFelsberg JRazis EDruschler KSchuller UWick WMohme MfalseThe clinical and molecular landscape of diffuse hemispheric glioma, H3 G34-mutant.<h4>Background</h4>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.<h4>Methods</h4>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.<h4>Results</h4>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 reveal2025-01-01T00:00:00Z2025 Jul2026-07-15T06:51:00.859Z2025-08-24T03:07:23.978ZS-EPMC123097183984293510.1093/neuonc/noaf015