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A comprehensive multicenter analysis of clinical, molecular, and imaging characteristics and outcomes of H3 K27-altered diffuse midline glioma in adults

Authors
 Yongsik Sim  ;  Andrew C McClelland  ;  Kaeum Choi  ;  Kyunghwa Han  ;  Yae Won Park  ;  Sung Soo Ahn  ;  Jong Hee Chang  ;  Se Hoon Kim  ;  Sharon Gardner  ;  Seung-Koo Lee  ;  Rajan Jain 
Citation
 JOURNAL OF NEUROSURGERY, Vol.142(5) : 1307-1318, 2025-01 
Journal Title
JOURNAL OF NEUROSURGERY
ISSN
 0022-3085 
Issue Date
2025-01
MeSH
1
Keywords
Adolescent ; Adult ; Aged ; Brain Neoplasms* / diagnostic imaging ; Brain Neoplasms* / genetics ; Brain Neoplasms* / mortality ; Brain Neoplasms* / pathology ; Brain Neoplasms* / surgery ; Child ; Female ; Glioma* / diagnostic imaging ; Glioma* / genetics ; Glioma* / mortality ; Glioma* / pathology ; Glioma* / surgery ; Histones* / genetics ; Histones* / metabolism ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Treatment Outcome ; Young Adult
Abstract
H3 K27 ; diffuse midline glioma ; magnetic resonance imaging ; survival ; tumor
Article Number
 10.3171/2024.8.JNS241180 
DOI
Objective: The objective was to comprehensively investigate the clinical, molecular, and imaging characteristics and outcomes of H3 K27-altered diffuse midline glioma (DMG) in adults. Methods: Retrospective chart and imaging reviews were performed in 111 adult patients with H3 K27-altered DMG from two tertiary institutions. Clinical, molecular, imaging, and survival characteristics were analyzed. Characteristics were compared between adult and 365 pediatric patients from a previous multicenter meta-analysis dataset. Cox analyses were performed to determine predictors of overall survival (OS) in adult patients. Results: The median (range) age of adult patients was 40 (18-75) years, and 64 males and 47 females were included. Adults had a higher male proportion (57.7% vs 45.3%, p = 0.023), lower proportion of histological grade 4 (41.4% vs 74.0%, p < 0.001), and different tumor locations (p < 0.001) compared with pediatric patients; adults commonly showed a thalamus location (41.5%) followed by the spinal cord (27.0%), whereas pediatric patients predominantly showed a pons location (64.9%). The OS of adults was longer than that of pediatric patients (30.3 vs 12.0 months, p < 0.001, log-rank test). Older age at diagnosis (HR 0.96, p = 0.001), histologically lower grade (HR 0.25, p = 0.003), and gross-total resection of nonenhancing tumor (HR 0.15, p = 0.003) were independent favorable prognostic factors. Conclusions: Adult patients with H3 K27-altered DMG showed distinct clinical, histological, and imaging characteristics compared to pediatric counterparts, with a significantly better prognosis. The authors' results suggest that aggressive surgery should be pursued when deemed feasible for better survival outcomes.
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Radiology (영상의학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Neurosurgery (신경외과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Se Hoon(김세훈) ORCID logo https://orcid.org/0000-0001-7516-7372
Park, Yae Won(박예원) ORCID logo https://orcid.org/0000-0001-8907-5401
Ahn, Sung Soo(안성수) ORCID logo https://orcid.org/0000-0002-0503-5558
Lee, Seung Koo(이승구) ORCID logo https://orcid.org/0000-0001-5646-4072
Chang, Jong Hee(장종희) ORCID logo https://orcid.org/0000-0003-1509-9800
Han, Kyung Hwa(한경화)
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/207526
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