Akeret, K; Padevit, L; Reifenberger, G; Von Deimling, A; Weller, M; Le Rhun, E; Bink, A; ... Sahm, F; + view all Akeret, K; Padevit, L; Reifenberger, G; Von Deimling, A; Weller, M; Le Rhun, E; Bink, A; Felsberg, J; Gramatzki, D; Brandner, S; Benhamida, JK; Wick, A; Tonn, JC; Mohme, M; Tabatabai, G; Capper, D; Snuderl, M; Razis, E; Ronellenfitsch, MW; Neidert, N; Ng, HK; Pohl, U; Bale, T; Quach, S; Rieger, D; Schüller, U; Onken, J; Drüschler, K; Maurage, CA; Regli, L; Healy, E; Graham, M; Hortobagyi, T; Paine, S; Bridges, L; Lausova, T; Medici, V; Sievers, P; Schrimpf, D; Wick, W; Sahm, F; - view fewer (2025) Anatomic Staging of H3 G34-Mutant Diffuse Hemispheric Glioma. Neurology , 105 (3) , Article e213861. 10.1212/WNL.0000000000213861.

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Abstract

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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