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

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

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。

Objective： To retrospectively summarize the clinicopathological features of epithelioid glioblastoma（Ep-GBM） and to explore new treatment for Ep-GBM. Methods： The clinical data of 13 patients with Ep-GBM, who were treated in our department from March 2016 to July 2017, were retrospectively analyzed. The clinicopathological features were summarized and the efficacy was evaluated. Results： The positive rate of BRAFV600 E 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 median progression-free time was 8.6（2.2–16.5） months. Three patients died and the 1-year overall survival rate was 54%. Conclusion： Ep-GBM has a high degree of malignancy and is prone to spread to leptomeninges. INI-1 expression and BRAFV600 E mutation are common for Ep-GBM. BRAF inhibitor might be a potential therapeutic drug for it.