Academic Journal
The clinical and molecular landscape of diffuse hemispheric glioma, H3 G34-mutant
| العنوان: | The clinical and molecular landscape of diffuse hemispheric glioma, H3 G34-mutant |
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| المؤلفون: | Le Rhun, Emilie, Bink, Andrea, Felsberg, Joerg, Gramatzki, Dorothee, Brandner, Sebastian, Benhamida, Jamal K, Wick, Antje, Tonn, Joerg C, Mohme, Malte, Tabatabai, Ghazaleh, Capper, David, Snuderl, Matija, Razis, Evangelia, Ronellenfitsch, Michael W, Neidert, Nicolas, Ng, Ho-Keung, Pohl, Ute, Bale, Tejus, Quach, Stefanie, Rieger, David, Schüller, Ulrich, Onken, Julia, Drüschler, Katharina, Maurage, Claude-Alain, Regli, Luca, Healy, Estelle, Graham, Maya, Hortobagyi, Tibor, Paine, Simon, Bridges, Leslie, Lausova, Tereza, Medici, Valentina, Sievers, Philipp, Schrimpf, David, Wick, Wolfgang, Sahm, Felix, Reifenberger, Guido, von Deimling, Andreas, Weller, Michael |
| المصدر: | Neuro-Oncology ; ISSN 1522-8517 1523-5866 |
| بيانات النشر: | Oxford University Press (OUP) |
| سنة النشر: | 2025 |
| الوصف: | Background Diffuse 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. Methods 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. Results 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. Conclusions 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. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| DOI: | 10.1093/neuonc/noaf015 |
| DOI: | 10.1093/neuonc/noaf015/61562110/noaf015.pdf |
| الاتاحة: | https://doi.org/10.1093/neuonc/noaf015 https://academic.oup.com/neuro-oncology/advance-article-pdf/doi/10.1093/neuonc/noaf015/61562110/noaf015.pdf |
| Rights: | https://creativecommons.org/licenses/by/4.0/ |
| رقم الانضمام: | edsbas.C979FA82 |
| قاعدة البيانات: | BASE |
| DOI: | 10.1093/neuonc/noaf015 |
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