Clinico-pathological analysis and review of the literature of diffuse hemispheric glioma,H3 G34-mutant
Lai Xuwen
Yan Guangning
Zeng Weipeng
He Wenyuan
Luo Jinxia
Wang Zhuocai
Wang Wei
Abstract：Objective To explore the clinicopathological characteristics of H3G34-mutant diffuse hemispheric glioma(DHG-G34),and analyze its immunophenotype,molecular characteristics and prognosis.Methods Review the data of 3 cases of DHG-G34,analyze their histological characteristics and immune phenotype under light microscopy,and evaluate the clinical and pathological features and prognosis of the tumor based on molecular changes and follow-up results.Results Two cases were located in the right frontal lobe,and one case involved multiple brain lobes including bilateral frontal lobe,left temporal lobe and basal ganglia.Preoperative imaging demonstrated cystic and solid masses in the cortex and border area in both cases of frontal lobe lesions,with enhanced scanning of the cyst wall and solid parts;Cace 3 shows irregular abnormal signal shadows in multiple cerebral lobes of the brain,with unclear enhancement.Histologically,two frontal lobe lesions manifested glioblastoma,and undifferentiated small cells densely distributed among GBM background.Case 3 involved multiple brain lobes and showed astrocytoma of grade 2-3.Immunohistochemical tests showed GFAP was diffusely expressed in all 3 cases,but olig-2,IDH1/2,ATRX,MGMT and BRAFV600E were negative in all cases.P53 was strongly positive in 1 case and completely lost in 2 cases(nonsense mutation).There was H3 G34R mutation in 2 cases and H3 G34V mutation in 1 case.Total resection was performed in case 1 and case 2,and one partial resection.Adjuvant radiotherapy and chemotherapy was not performed in case 2,and the tumor recurred rapidly after 3 months.Case 1 and 3 were treated with radiotherapy and chemotherapy after operation,and the tumor progressed at 12 and 17 months respectively after surgery.Conclusions Diffuse hemispheric glioma,H3 G34-mutant is an infiltrative glioma involving the cerebral hemispheres that is more likely to occur in children and adolescents.DHG-G34 has characteristic immunophenotype and molecular changes despite of its morphological diversity.The DHG-G34 patients'prognosis is poor,but superior to IDH wild-type GBM and diffuse midline glioma(H3K27 variant).
Keywords：gliomaH3 G34-mutanthistologymolecular pathologyprognosis
Publication Date：2023-12-30
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：6( 46-51 )