Oki Sogo, Yamaguchi Shigeru, Ishi Yukitomo, Motegi Hiroaki, Gekka Masayuki, Tanei Zen-Ichi, Kanno-Okada Hiromi, Takakuwa Emi, Tanaka Satoshi, Mori Takashi, Nishioka Kentaro, Aoyama Hidefumi, Tanaka Shinya, Fujimura Miki
Department of Neurosurgery, Hokkaido University Graduate School of Medicine, Kita 15 Nishi 7, Kita-Ku, Sapporo, Hokkaido, Japan.
Department of Neurosurgery, Sapporo Azabu Neurosurgical Hospital, Sapporo, Japan.
Acta Neurochir (Wien). 2025 Aug 8;167(1):217. doi: 10.1007/s00701-025-06633-1.
Cerebellar glioblastoma in adults is a rare brain tumor with poor outcomes. This study aimed to assess the clinical characteristics, genetic features, and prognosis of a series of cerebellar glioblastomas, with special attention to their recurrence patterns.
We retrospectively analyzed patients who underwent treatment between 2008 and 2023. The patient characteristics, treatment methods, genetic features, and prognoses were assessed.
Among 274 cases of histological glioblastomas, eleven patients with cerebellar glioblastomas were identified. Pathological results revealed microvascular proliferation and/or necrosis in all cases. All patients underwent surgery and local radiotherapy combined with temozolomide chemotherapy. The median progression-free survival and overall survival were 15.3 months and 22.8 months, respectively. Subventricular zone involvement was recognized in ten patients. Of the eight patients who experienced recurrence, seven had distant or disseminated recurrence, and only one experienced local recurrence. None of the tumors harbored mutations in the IDH1/2, H3F3A, or TERT promoters. Three patients showed negative expression of O-methylguanine DNA methyltransferase (MGMT), and four patients showed positive expression.
In our cohort, cerebellar glioblastomas showed clinical characteristics such as recurrence patterns and genetic features such as IDH, H3F3A, and TERT promoter regions that differed from those of typical supratentorial gliomas. Further studies are necessary to fully elucidate the clinical characteristics.
成人小脑胶质母细胞瘤是一种罕见的脑肿瘤，预后较差。本研究旨在评估一系列小脑胶质母细胞瘤的临床特征、基因特征和预后，特别关注其复发模式。
我们回顾性分析了2008年至2023年期间接受治疗的患者。评估了患者的特征、治疗方法、基因特征和预后。
在274例组织学确诊的胶质母细胞瘤病例中，确定了11例小脑胶质母细胞瘤患者。病理结果显示所有病例均有微血管增生和/或坏死。所有患者均接受了手术、局部放疗联合替莫唑胺化疗。无进展生存期和总生存期的中位数分别为15.3个月和22.8个月。10例患者被发现有脑室下区受累。在8例复发的患者中，7例有远处或播散性复发，只有1例有局部复发。所有肿瘤均未在异柠檬酸脱氢酶1/2（IDH1/2）、组蛋白H3第3A变异体（H3F3A）或端粒酶逆转录酶（TERT）启动子中发生突变。3例患者显示O6-甲基鸟嘌呤-DNA甲基转移酶（MGMT）阴性表达，4例患者显示阳性表达。
在我们的队列中，小脑胶质母细胞瘤表现出与典型幕上胶质瘤不同的复发模式等临床特征以及异柠檬酸脱氢酶、组蛋白H3第3A变异体和端粒酶逆转录酶启动子区域等基因特征。需要进一步研究以充分阐明其临床特征。
