h3g34突变体弥漫性半球胶质瘤的临床病理特征

IF 0.7 4区 医学 Q4 PATHOLOGY
Fetal and Pediatric Pathology Pub Date : 2025-03-01 Epub Date: 2025-02-28 DOI:10.1080/15513815.2025.2460560
Juan Cao, Yongxian Chen, Guocheng Yang, Xiaoxiao He, Hailun Chen, Zaoling Luo, Jingting Liao, Zhihui Huang, Weiguo Cao
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引用次数: 0

摘要

目的:探讨3例弥漫性半球胶质瘤H3G34突变体(H3G34 DHG)的临床表现、影像学特征及病理特点。方法:本研究采用单中心回顾性队列方法分析45例小儿型弥漫性高级别胶质瘤病例。结果:在组织学上,病例1和病例2为胶质母细胞瘤结构,病例3为原始神经外胚层肿瘤(PNET)形态,伴有神经节细胞分化。免疫组化染色显示3例肿瘤细胞中H3G34R、P53弥漫性表达,而Olig2、ATRX、IDH1/2、BRAF V600E均未表达。结论:H3 G34 DHG在年轻患者中发生率更高,且几乎所有病变均位于大脑半球。MRI显示肿块效应、水肿及轻度强化。组织学表现为胶质母细胞瘤结构和PNET形态。免疫组化结果显示,PNET形态学中H3G34R的表达更为显著。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Clinicopathological Features of Diffuse Hemispheric Glioma, H3G34-Mutant.

Objective: To investigate the clinical manifestations, radiographic features, and pathological characteristics of three cases of diffuse hemispheric gliomas, H3G34-mutant (H3G34 DHG).

Methods: This study used a single-center retrospective cohort approach to analyze 45 pediatric-type diffuse high-grade glioma cases.

Results: Histologically, case 1 and case 2 had glioblastoma structures, and case 3 had primitive neuroectodermal tumor (PNET) morphology with ganglion cell differentiation. Immunohistochemical staining revealed diffuse expression of H3G34R and P53, but no expression of Olig2, ATRX, IDH1/2 and BRAF V600E in tumor cells in all three cases.

Conclusions: H3 G34 DHG occurs more significantly in younger patients, and nearly all lesions are located in the cerebral hemisphere. MRI showed mass effects, edema and mild enhancement. The histological type showed glioblastoma structure and PNET morphology. Immunohistochemistry showed that the expression of H3G34R was more significant in PNET morphology.

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来源期刊
CiteScore
3.00
自引率
0.00%
发文量
68
审稿时长
6-12 weeks
期刊介绍: Fetal and Pediatric Pathology is an established bimonthly international journal that publishes data on diseases of the developing embryo, newborns, children, and adolescents. The journal publishes original and review articles and reportable case reports. The expanded scope of the journal encompasses molecular basis of genetic disorders; molecular basis of diseases that lead to implantation failures; molecular basis of abnormal placentation; placentology and molecular basis of habitual abortion; intrauterine development and molecular basis of embryonic death; pathogenisis and etiologic factors involved in sudden infant death syndrome; the underlying molecular basis, and pathogenesis of diseases that lead to morbidity and mortality in newborns; prenatal, perinatal, and pediatric diseases and molecular basis of diseases of childhood including solid tumors and tumors of the hematopoietic system; and experimental and molecular pathology.
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