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000302835 1001_ $$00000-0002-5946-4999$$aAkeret, Kevin$$b0
000302835 245__ $$aAnatomic Staging of H3 G34-Mutant Diffuse Hemispheric Glioma.
000302835 260__ $$aPhiladelphia, Pa.$$bWolters Kluwer$$c2025
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000302835 520__ $$aH3 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.
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000302835 650_7 $$2NLM Chemicals$$aHistones
000302835 650_7 $$0EC 2.1.1.-$$2NLM Chemicals$$aDNA Modification Methylases
000302835 650_2 $$2MeSH$$aHumans
000302835 650_2 $$2MeSH$$aFemale
000302835 650_2 $$2MeSH$$aMale
000302835 650_2 $$2MeSH$$aGlioma: genetics
000302835 650_2 $$2MeSH$$aGlioma: pathology
000302835 650_2 $$2MeSH$$aGlioma: diagnostic imaging
000302835 650_2 $$2MeSH$$aGlioma: mortality
000302835 650_2 $$2MeSH$$aBrain Neoplasms: genetics
000302835 650_2 $$2MeSH$$aBrain Neoplasms: pathology
000302835 650_2 $$2MeSH$$aBrain Neoplasms: diagnostic imaging
000302835 650_2 $$2MeSH$$aBrain Neoplasms: mortality
000302835 650_2 $$2MeSH$$aAdult
000302835 650_2 $$2MeSH$$aYoung Adult
000302835 650_2 $$2MeSH$$aMutation: genetics
000302835 650_2 $$2MeSH$$aHistones: genetics
000302835 650_2 $$2MeSH$$aNeoplasm Staging
000302835 650_2 $$2MeSH$$aAdolescent
000302835 650_2 $$2MeSH$$aMiddle Aged
000302835 650_2 $$2MeSH$$aPrognosis
000302835 650_2 $$2MeSH$$aCohort Studies
000302835 650_2 $$2MeSH$$aKaplan-Meier Estimate
000302835 650_2 $$2MeSH$$aDNA Modification Methylases: genetics
000302835 7001_ $$00000-0003-2461-0404$$aPadevit, Luis$$b1
000302835 7001_ $$0P:(DE-HGF)0$$aReifenberger, Guido$$b2
000302835 7001_ $$0P:(DE-He78)a8a10626a848d31e70cfd96a133cc144$$avon Deimling, Andreas$$b3$$udkfz
000302835 7001_ $$00000-0002-1748-174X$$aWeller, Michael$$b4
000302835 7001_ $$00000-0002-9408-3278$$aLe Rhun, Emilie$$b5
000302835 7001_ $$aGroup, H3 G34 DHG Study$$b6$$eCollaboration Author
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