WHO(2016)中枢神经系统肿瘤新增分类上皮样胶质母细胞瘤的临床病理特征

目的：回顾性分析上皮样胶质母细胞瘤(epithelioid glioblastoma，Ep-GBM)的临床病理特征，探索Ep-GBM的临床诊疗新思路。方法：回顾性分析2016年3月至2017年7月广东三九脑科医院肿瘤科收治的13例Ep-GBM患者临床资料，对其临床病理特征进行总结，并对其疗效进行评估。结果：13例患者的分子病理学检测发现BRAFV600E阳性率76.9%(10/13)，INI-1阳性率80%(8/10)，中位Ki-67指数30%。病程中出现脑膜和/或脊膜转移9例(69.7%)。中位随访时间12(6~25)个月。中位无进展生存期为8.6(2.2~16.5)个月，3例患者死亡，1年生存率为54%。结论：Ep-GBM恶性程度高，容易发生脑膜及脊膜播散。在初次诊断时应重视全中枢影像评估以决定是否需全中枢放射治疗。Ep-GBM常伴有INI-1表达及BRA FV600E突变，BRA F抑制剂是一种潜在的治疗药物。

Clinicopathological features for epithelioid glioblastoma: A newly defined tumor by the 2016 World Health Organization Classification of Tumors of the Central Nervous System

Objective: To retrospectively summarize the clinicopathological features of epithelioidglioblastoma (Ep-GBM) and to explore new treatment for Ep-GBM.Methods: Th e clinical data of 13 patients with Ep-GBM, who were treated in our department fromMarch 2016 to July 2017, were retrospectively analyzed. The clinicopathological features weresummarized and the efficacy was evaluated.Results: The positive rate of BRAFV600E mutant and INI-1 was 76.9% (10/13) and 80% (8/10),respectively, while the median Ki-67 index was 30%. Meningeal metastases occurred in 9 cases(69.7%) during the course. The median follow-up time was 12 (6–25) months, and the medianprogression-free time was 8.6 (2.2–16.5) months. Three patients died and the 1-year overall survivalrate was 54%.Conclusion: Ep-GBM has a high degree of malignancy and is prone to spread to leptomeninges.INI-1 expression and BRAFV600E mutation are common for Ep-GBM. BRAF inhibitor might be apotential therapeutic drug for it.