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| Home > Publications database > Anatomic Staging of H3 G34-Mutant Diffuse Hemispheric Glioma. |
| Home > Publications database > Anatomic Staging of H3 G34-Mutant Diffuse Hemispheric Glioma. |
| Journal Article | DKFZ-2025-01375 |
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2025
Wolters Kluwer
Philadelphia, Pa.
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Please use a persistent id in citations: doi:10.1212/WNL.0000000000213861
Abstract: 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.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.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).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.
Keyword(s): Humans (MeSH) ; Female (MeSH) ; Male (MeSH) ; Glioma: genetics (MeSH) ; Glioma: pathology (MeSH) ; Glioma: diagnostic imaging (MeSH) ; Glioma: mortality (MeSH) ; Brain Neoplasms: genetics (MeSH) ; Brain Neoplasms: pathology (MeSH) ; Brain Neoplasms: diagnostic imaging (MeSH) ; Brain Neoplasms: mortality (MeSH) ; Adult (MeSH) ; Young Adult (MeSH) ; Mutation: genetics (MeSH) ; Histones: genetics (MeSH) ; Neoplasm Staging (MeSH) ; Adolescent (MeSH) ; Middle Aged (MeSH) ; Prognosis (MeSH) ; Cohort Studies (MeSH) ; Kaplan-Meier Estimate (MeSH) ; DNA Modification Methylases: genetics (MeSH) ; Histones ; DNA Modification Methylases
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