Anatomic staging of H3 G34-mutant diffuse hemispheric glioma
Objectives - H3 G34-mutant diffuse hemispheric gliomas are rare, aggressive primary brain tumors predominantly affecting young patients. We investigated the prognostic value of anatomic staging (AS)—a system previously validated in adult-type diffuse gliomas—in this molecularly distinct tumor type....
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| Main Authors: | , , , , , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
July 10, 2025
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| In: |
Neurology
Year: 2025, Volume: 105, Issue: 3 |
| ISSN: | 1526-632X |
| DOI: | 10.1212/WNL.0000000000213861 |
| Online Access: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1212/WNL.0000000000213861 Verlag, lizenzpflichtig, Volltext: https://www.neurology.org/doi/10.1212/WNL.0000000000213861 
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| Author Notes: | Kevin Akeret, Luis Padevit, Guido Reifenberger, Andreas von Deimling, Michael Weller, and Emilie Le Rhun, for the H3 G34 DHG Study Group |
| Summary: | Objectives - H3 G34-mutant diffuse hemispheric gliomas are rare, aggressive primary brain tumors predominantly affecting young patients. We investigated the prognostic value of anatomic staging (AS)—a system previously validated in adult-type diffuse gliomas—in this molecularly distinct tumor type. - Methods - Patients from an international cohort with H3 G34-mutant gliomas underwent AS based on pretreatment imaging, performed independently by 2 raters blinded to clinical outcomes. Inter-rater reliability was evaluated using Cohen kappa. Kaplan-Meier curves and Cox proportional hazards models—unadjusted and adjusted for sex, extent of resection, and O6-methylguanine DNA-methyltransferase (MGMT) status—were used to analyze overall survival across stages. - Results - Thirty-seven patients were included (median age 22 years; 54% female). Inter-rater reliability was high (weighted κ = 0.93, 95% CI 0.85-1.0). Median overall survival was 36 months for stage 1 (95% CI 16-67 months), 25 months for stage 2 (95% CI 8-41), and 9 months for stage 3 (95% CI 3-26). After adjustment for sex, extent of resection, and MGMT status, survival differences persisted (hazard ratio [HR]Stage 2 adjusted 2.25, 95% CI 0.67-7.5, p = 0.19; HRStage 3 adjusted 4.37, 95% CI 1.39-13.7, p = 0.01). - Discussion - AS is reproducible and prognostically relevant for H3 G34-mutant gliomas, providing insights into tumor spread. It may inform treatment decisions, but larger studies are needed to confirm its clinical utility. |
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| Item Description: | Gesehen am 15.12.2025 |
| Physical Description: | Online Resource |
| ISSN: | 1526-632X |
| DOI: | 10.1212/WNL.0000000000213861 |