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@ARTICLE{Akeret:302835,
      author       = {K. Akeret and L. Padevit and G. Reifenberger$^*$ and A. von
                      Deimling$^*$ and M. Weller and E. Le Rhun},
      collaboration = {H. G. D. S. Group},
      title        = {{A}natomic {S}taging of {H}3 {G}34-{M}utant {D}iffuse
                      {H}emispheric {G}lioma.},
      journal      = {Neurology},
      volume       = {105},
      number       = {3},
      issn         = {0028-3878},
      address      = {Philadelphia, Pa.},
      publisher    = {Wolters Kluwer},
      reportid     = {DKFZ-2025-01375},
      pages        = {e213861},
      year         = {2025},
      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.},
      keywords     = {Humans / Female / Male / Glioma: genetics / Glioma:
                      pathology / Glioma: diagnostic imaging / Glioma: mortality /
                      Brain Neoplasms: genetics / Brain Neoplasms: pathology /
                      Brain Neoplasms: diagnostic imaging / Brain Neoplasms:
                      mortality / Adult / Young Adult / Mutation: genetics /
                      Histones: genetics / Neoplasm Staging / Adolescent / Middle
                      Aged / Prognosis / Cohort Studies / Kaplan-Meier Estimate /
                      DNA Modification Methylases: genetics / Histones (NLM
                      Chemicals) / DNA Modification Methylases (NLM Chemicals)},
      cin          = {B300 / HD01 / ED01},
      ddc          = {610},
      cid          = {I:(DE-He78)B300-20160331 / I:(DE-He78)HD01-20160331 /
                      I:(DE-He78)ED01-20160331},
      pnm          = {312 - Funktionelle und strukturelle Genomforschung
                      (POF4-312)},
      pid          = {G:(DE-HGF)POF4-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:40638885},
      doi          = {10.1212/WNL.0000000000213861},
      url          = {https://inrepo02.dkfz.de/record/302835},
}