000298178 001__ 298178
000298178 005__ 20250831022343.0
000298178 0247_ $$2doi$$a10.1093/neuonc/noaf015
000298178 0247_ $$2pmid$$apmid:39842935
000298178 0247_ $$2ISSN$$a1522-8517
000298178 0247_ $$2ISSN$$a1523-5866
000298178 0247_ $$2altmetric$$aaltmetric:173497445
000298178 037__ $$aDKFZ-2025-00197
000298178 041__ $$aEnglish
000298178 082__ $$a610
000298178 1001_ $$00000-0002-9408-3278$$aLe Rhun, Emilie$$b0
000298178 245__ $$aThe clinical and molecular landscape of diffuse hemispheric glioma, H3 G34-mutant.
000298178 260__ $$aOxford$$bOxford Univ. Press$$c2025
000298178 3367_ $$2DRIVER$$aarticle
000298178 3367_ $$2DataCite$$aOutput Types/Journal article
000298178 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1756374748_18474
000298178 3367_ $$2BibTeX$$aARTICLE
000298178 3367_ $$2ORCID$$aJOURNAL_ARTICLE
000298178 3367_ $$00$$2EndNote$$aJournal Article
000298178 500__ $$a#LA:B300# / Volume 27, Issue 6, June 2025, Pages 1519–1535
000298178 520__ $$aDiffuse hemispheric glioma, histone 3 (H3) G34-mutant, has been newly defined in the 2021 WHO classification of central nervous system tumors. Here we sought to define the prognostic roles of clinical, neuroimaging, pathological, and molecular features of these tumors.We retrospectively assembled a cohort of 114 patients (median age 22 years) with diffuse hemispheric glioma, H3 G34-mutant, CNS WHO grade 4 and profiled the imaging, histological and molecular landscape of their tumors.Compared with glioblastoma, H3 G34-mutant diffuse hemispheric gliomas exhibited less avid contrast enhancement, necrosis and edema on MRI. Comprehensive analyses of mutational and DNA copy number profiles revealed recurrent mutations in TP53 and ATRX, homozygous deletions of CDKN2A/B, and amplifications of PDGFRA, EGFR, CCND2, and MYCN. MGMT promoter methylation was detected in 79 tumors (75%); 11 tumors (13%) showed DNA copy number profiles suggestive of circumscribed deletions on 10q26.3 involving the MGMT locus. Median survival was 21.5 months. Female sex, gross total resection, and MGMT promoter methylation were positive prognostic factors on univariate analysis. Among radiological, pathological and molecular features, absence of pial invasion, and presence of microvascular proliferation and CDK6 amplification were positive prognostic factors on univariate analyses.This study refines the clinical and molecular landscape of H3 G34-mutant diffuse hemispheric gliomas. Dedicated trials for this novel tumor type are urgently needed.
000298178 536__ $$0G:(DE-HGF)POF4-312$$a312 - Funktionelle und strukturelle Genomforschung (POF4-312)$$cPOF4-312$$fPOF IV$$x0
000298178 588__ $$aDataset connected to CrossRef, PubMed, , Journals: inrepo02.dkfz.de
000298178 650_7 $$2Other$$aMGMT
000298178 650_7 $$2Other$$aGlioblastoma
000298178 650_7 $$2Other$$ahistone
000298178 650_7 $$2Other$$aloss
000298178 650_7 $$2Other$$amethylation
000298178 7001_ $$aBink, Andrea$$b1
000298178 7001_ $$0P:(DE-HGF)0$$aFelsberg, Joerg$$b2
000298178 7001_ $$00000-0002-9054-5498$$aGramatzki, Dorothee$$b3
000298178 7001_ $$00000-0002-9821-0342$$aBrandner, Sebastian$$b4
000298178 7001_ $$aBenhamida, Jamal K$$b5
000298178 7001_ $$0P:(DE-HGF)0$$aWick, Antje$$b6
000298178 7001_ $$0P:(DE-HGF)0$$aTonn, Joerg C$$b7
000298178 7001_ $$00000-0003-0627-9092$$aMohme, Malte$$b8
000298178 7001_ $$0P:(DE-He78)d53ff17be4b468909d803eccd92df3d6$$aTabatabai, Ghazaleh$$b9$$udkfz
000298178 7001_ $$0P:(DE-He78)51bf9ae9cb5771b30c483e5597ef606c$$aCapper, David$$b10$$udkfz
000298178 7001_ $$00000-0003-0752-0917$$aSnuderl, Matija$$b11
000298178 7001_ $$aRazis, Evangelia$$b12
000298178 7001_ $$aRonellenfitsch, Michael W$$b13
000298178 7001_ $$aNeidert, Nicolas$$b14
000298178 7001_ $$aNg, Ho-Keung$$b15
000298178 7001_ $$aPohl, Ute$$b16
000298178 7001_ $$aBale, Tejus$$b17
000298178 7001_ $$aQuach, Stefanie$$b18
000298178 7001_ $$aRieger, David$$b19
000298178 7001_ $$00000-0002-8731-1121$$aSchüller, Ulrich$$b20
000298178 7001_ $$0P:(DE-HGF)0$$aOnken, Julia$$b21
000298178 7001_ $$0P:(DE-HGF)0$$aDrüschler, Katharina$$b22
000298178 7001_ $$aMaurage, Claude-Alain$$b23
000298178 7001_ $$00000-0003-4639-4474$$aRegli, Luca$$b24
000298178 7001_ $$aHealy, Estelle$$b25
000298178 7001_ $$aGraham, Maya$$b26
000298178 7001_ $$00000-0001-5732-7942$$aHortobagyi, Tibor$$b27
000298178 7001_ $$00000-0002-1434-3931$$aPaine, Simon$$b28
000298178 7001_ $$aBridges, Leslie$$b29
000298178 7001_ $$0P:(DE-He78)5497d0db009ca23fed3a604a72280bb3$$aLausova, Tereza$$b30$$udkfz
000298178 7001_ $$aMedici, Valentina$$b31
000298178 7001_ $$0P:(DE-He78)8aad075b17d93a5636a34942bdbd7ee6$$aSievers, Philipp$$b32$$udkfz
000298178 7001_ $$0P:(DE-HGF)0$$aSchrimpf, David$$b33
000298178 7001_ $$0P:(DE-He78)92e9783ca7025f36ce14e12cd348d2ee$$aWick, Wolfgang$$b34$$udkfz
000298178 7001_ $$0P:(DE-He78)a1f4b408b9155beb2a8f7cba4d04fe88$$aSahm, Felix$$b35$$udkfz
000298178 7001_ $$00000-0002-1419-9837$$aReifenberger, Guido$$b36
000298178 7001_ $$0P:(DE-He78)a8a10626a848d31e70cfd96a133cc144$$avon Deimling, Andreas$$b37$$eLast author$$udkfz
000298178 7001_ $$00000-0002-1748-174X$$aWeller, Michael$$b38
000298178 7001_ $$aGroup, H3 G34 DHG Study$$b39$$eCollaboration Author
000298178 773__ $$0PERI:(DE-600)2094060-9$$a10.1093/neuonc/noaf015$$gp. noaf015$$n6$$p1519–1535$$tNeuro-Oncology$$v27$$x1522-8517$$y2025
000298178 909CO $$ooai:inrepo02.dkfz.de:298178$$pVDB
000298178 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-HGF)0$$aDeutsches Krebsforschungszentrum$$b2$$kDKFZ
000298178 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-HGF)0$$aDeutsches Krebsforschungszentrum$$b6$$kDKFZ
000298178 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-HGF)0$$aDeutsches Krebsforschungszentrum$$b7$$kDKFZ
000298178 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)d53ff17be4b468909d803eccd92df3d6$$aDeutsches Krebsforschungszentrum$$b9$$kDKFZ
000298178 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)51bf9ae9cb5771b30c483e5597ef606c$$aDeutsches Krebsforschungszentrum$$b10$$kDKFZ
000298178 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-HGF)0$$aDeutsches Krebsforschungszentrum$$b21$$kDKFZ
000298178 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-HGF)0$$aDeutsches Krebsforschungszentrum$$b22$$kDKFZ
000298178 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)5497d0db009ca23fed3a604a72280bb3$$aDeutsches Krebsforschungszentrum$$b30$$kDKFZ
000298178 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)8aad075b17d93a5636a34942bdbd7ee6$$aDeutsches Krebsforschungszentrum$$b32$$kDKFZ
000298178 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-HGF)0$$aDeutsches Krebsforschungszentrum$$b33$$kDKFZ
000298178 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)92e9783ca7025f36ce14e12cd348d2ee$$aDeutsches Krebsforschungszentrum$$b34$$kDKFZ
000298178 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)a1f4b408b9155beb2a8f7cba4d04fe88$$aDeutsches Krebsforschungszentrum$$b35$$kDKFZ
000298178 9101_ $$0I:(DE-588b)2036810-0$$60000-0002-1419-9837$$aDeutsches Krebsforschungszentrum$$b36$$kDKFZ
000298178 9101_ $$0I:(DE-588b)2036810-0$$6P:(DE-He78)a8a10626a848d31e70cfd96a133cc144$$aDeutsches Krebsforschungszentrum$$b37$$kDKFZ
000298178 9131_ $$0G:(DE-HGF)POF4-312$$1G:(DE-HGF)POF4-310$$2G:(DE-HGF)POF4-300$$3G:(DE-HGF)POF4$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lKrebsforschung$$vFunktionelle und strukturelle Genomforschung$$x0
000298178 9141_ $$y2025
000298178 915__ $$0StatID:(DE-HGF)0420$$2StatID$$aNationallizenz$$d2024-12-11$$wger
000298178 915__ $$0StatID:(DE-HGF)0100$$2StatID$$aJCR$$bNEURO-ONCOLOGY : 2022$$d2024-12-11
000298178 915__ $$0StatID:(DE-HGF)0200$$2StatID$$aDBCoverage$$bSCOPUS$$d2024-12-11
000298178 915__ $$0StatID:(DE-HGF)0300$$2StatID$$aDBCoverage$$bMedline$$d2024-12-11
000298178 915__ $$0StatID:(DE-HGF)0199$$2StatID$$aDBCoverage$$bClarivate Analytics Master Journal List$$d2024-12-11
000298178 915__ $$0StatID:(DE-HGF)0160$$2StatID$$aDBCoverage$$bEssential Science Indicators$$d2024-12-11
000298178 915__ $$0StatID:(DE-HGF)1110$$2StatID$$aDBCoverage$$bCurrent Contents - Clinical Medicine$$d2024-12-11
000298178 915__ $$0StatID:(DE-HGF)0113$$2StatID$$aWoS$$bScience Citation Index Expanded$$d2024-12-11
000298178 915__ $$0StatID:(DE-HGF)0150$$2StatID$$aDBCoverage$$bWeb of Science Core Collection$$d2024-12-11
000298178 915__ $$0StatID:(DE-HGF)9915$$2StatID$$aIF >= 15$$bNEURO-ONCOLOGY : 2022$$d2024-12-11
000298178 9202_ $$0I:(DE-He78)B300-20160331$$kB300$$lKKE Neuropathologie$$x0
000298178 9201_ $$0I:(DE-He78)ED01-20160331$$kED01$$lDKTK Koordinierungsstelle Essen/Düsseldorf$$x0
000298178 9201_ $$0I:(DE-He78)B320-20160331$$kB320$$lKKE Neuroonkologie$$x1
000298178 9201_ $$0I:(DE-He78)MU01-20160331$$kMU01$$lDKTK Koordinierungsstelle München$$x2
000298178 9201_ $$0I:(DE-He78)TU01-20160331$$kTU01$$lDKTK Koordinierungsstelle Tübingen$$x3
000298178 9201_ $$0I:(DE-He78)BE01-20160331$$kBE01$$lDKTK Koordinierungsstelle Berlin$$x4
000298178 9201_ $$0I:(DE-He78)B300-20160331$$kB300$$lKKE Neuropathologie$$x5
000298178 9201_ $$0I:(DE-He78)HD01-20160331$$kHD01$$lDKTK HD zentral$$x6
000298178 980__ $$ajournal
000298178 980__ $$aVDB
000298178 980__ $$aI:(DE-He78)ED01-20160331
000298178 980__ $$aI:(DE-He78)B320-20160331
000298178 980__ $$aI:(DE-He78)MU01-20160331
000298178 980__ $$aI:(DE-He78)TU01-20160331
000298178 980__ $$aI:(DE-He78)BE01-20160331
000298178 980__ $$aI:(DE-He78)B300-20160331
000298178 980__ $$aI:(DE-He78)HD01-20160331
000298178 980__ $$aUNRESTRICTED