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100 1 _ |a Akeret, Kevin
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245 _ _ |a Anatomic Staging of H3 G34-Mutant Diffuse Hemispheric Glioma.
260 _ _ |a Philadelphia, Pa.
|c 2025
|b Wolters Kluwer
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520 _ _ |a 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.
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650 _ 7 |a Histones
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650 _ 7 |a DNA Modification Methylases
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650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a Female
|2 MeSH
650 _ 2 |a Male
|2 MeSH
650 _ 2 |a Glioma: genetics
|2 MeSH
650 _ 2 |a Glioma: pathology
|2 MeSH
650 _ 2 |a Glioma: diagnostic imaging
|2 MeSH
650 _ 2 |a Glioma: mortality
|2 MeSH
650 _ 2 |a Brain Neoplasms: genetics
|2 MeSH
650 _ 2 |a Brain Neoplasms: pathology
|2 MeSH
650 _ 2 |a Brain Neoplasms: diagnostic imaging
|2 MeSH
650 _ 2 |a Brain Neoplasms: mortality
|2 MeSH
650 _ 2 |a Adult
|2 MeSH
650 _ 2 |a Young Adult
|2 MeSH
650 _ 2 |a Mutation: genetics
|2 MeSH
650 _ 2 |a Histones: genetics
|2 MeSH
650 _ 2 |a Neoplasm Staging
|2 MeSH
650 _ 2 |a Adolescent
|2 MeSH
650 _ 2 |a Middle Aged
|2 MeSH
650 _ 2 |a Prognosis
|2 MeSH
650 _ 2 |a Cohort Studies
|2 MeSH
650 _ 2 |a Kaplan-Meier Estimate
|2 MeSH
650 _ 2 |a DNA Modification Methylases: genetics
|2 MeSH
700 1 _ |a Padevit, Luis
|0 0000-0003-2461-0404
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700 1 _ |a Reifenberger, Guido
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700 1 _ |a von Deimling, Andreas
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700 1 _ |a Weller, Michael
|0 0000-0002-1748-174X
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700 1 _ |a Le Rhun, Emilie
|0 0000-0002-9408-3278
|b 5
700 1 _ |a Group, H3 G34 DHG Study
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773 _ _ |a 10.1212/WNL.0000000000213861
|g Vol. 105, no. 3, p. e213861
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