Yuzawa Sayaka, Kanemura Yonehiro, Hayashi Manami, Kamikokura Yuki, Kinoshita Manabu, Tanino Mishie
Department of Diagnostic Pathology, Asahikawa Medical University Hospital, Asahikawa, Japan.
Department of Biomedical Research and Innovation, Institute for Clinical Research, NHO Osaka National Hospital, Osaka, Japan.
Neuropathology. 2025 Aug;45(4):e70022. doi: 10.1111/neup.70022.
Diffuse hemispheric glioma, H3 G34-mutant (DHG), is a newly defined pediatric-type tumor in the 2021 WHO classification of central nervous system (CNS) tumors. DHGs harbor missense mutations at codon 35 of H3F3A (H3.3 G35R/V mutations) and exhibit diverse histopathological features, such as glioblastomas or CNS embryonal tumors. Regardless of histological variation, they demonstrate uniform immunohistochemical and molecular findings: Olig2 negative, ATRX loss, p53 positive, and MGMT promoter methylated. These tumors occur predominantly in the cerebral hemispheres of adolescents and young adults, while they are extremely rare in middle-aged and elderly individuals. Here, we report a middle-aged case of DHG with prominent perivascular invasion. The tumor initially demonstrated a focal glioblastoma-like area, whereas the recurrent tumor predominantly showed perivascular spread of spindle cells. We reviewed previously reported DHG cases in middle-aged and elderly patients and compared their clinicopathological features with those of adolescents and young adults.
弥漫性半球胶质瘤,H3 G34突变型(DHG),是2021年世界卫生组织中枢神经系统(CNS)肿瘤分类中新定义的一种儿童型肿瘤。DHG在H3F3A的第35密码子处存在错义突变(H3.3 G35R/V突变),并表现出多种组织病理学特征,如胶质母细胞瘤或中枢神经系统胚胎性肿瘤。无论组织学变异如何,它们都表现出一致的免疫组化和分子特征:Olig2阴性、ATRX缺失、p53阳性和MGMT启动子甲基化。这些肿瘤主要发生在青少年和年轻成年人的大脑半球,而在中年和老年个体中极为罕见。在此,我们报告一例具有显著血管周围浸润的中年DHG病例。肿瘤最初表现为局灶性胶质母细胞瘤样区域,而复发肿瘤主要表现为梭形细胞的血管周围扩散。我们回顾了先前报道的中年和老年患者的DHG病例,并将其临床病理特征与青少年和年轻成年人的进行了比较。