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

摘要

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BRAFV600E突变,BRAF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 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 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 BRAFV600E mutation are common for Ep-GBM.BRAF inhibitor might be a potential therapeutic drug for it.