上皮样胶质母细胞瘤临床病理与影像特征研究  

Analysis of clinical, pathological and radiological features of epithelioid glioblastoma

作　　者：娄平阳[1] 蒋海辉[2] 郭杨 林松[2] Lou Pingyang;Jiang Haihui;Guo Yang;Lin Song(Department of Neurosurgery, Henan Provincial People’s Hospital, Zhengzhou 450003, China;Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China)

机构地区：[1]河南省人民医院神经外科,郑州450003 [2]首都医科大学附属北京天坛医院神经外科,100070

出　　处：《中华实验外科杂志》2019年第4期762-764,共3页Chinese Journal of Experimental Surgery

摘　　要：目的探讨上皮样胶质母细胞瘤(Ep-GBM)的临床、病理及影像学特征。方法分析河南省人民医院神经外科收治的20例Ep-GBM患者的临床资料,对其临床、病理及影像特征进行归纳总结。结果Ep-GBM患者发病年龄为(43.2±19.7)岁,年龄范围4~70岁。影像学上可见明显强化及坏死灶,约40.0%患者合并嚢变;灌注加权成像呈高灌注表现,波谱分析提示肿瘤区域N-乙酰天门冬氨酸(NAA)峰下降,胆碱(Cho)峰升高。分子病理学检测发现BRAF V600E阳性率33.3%,整合酶相互作用分子(INI-l)阳性率87.5%,中位细胞细胞核增殖抗原(Ki-67)为50%,异柠檬酸脱氢酶(IDH)突变率及染色体1p/19q缺失率为0%。本组患者中位无进展生存期为8.0个月,中位总体生存期为15.0个月。生存分析显示肿瘤未全切及端粒酶反转录酶(TERT)突变是预后危险因素。结论Ep-GBM作为一种新胶质母细胞瘤亚型,其预后不良,肿瘤全切是其治疗的关键;TERT突变是提示Ep-GBM预后极差的分子标志物。Objective To explore the clinical, pathological and radiological features of epithelioid glioblastoma (Ep-GBM). Methods A retrospective analysis of 20 patients’ clinical data who were treated and diagnosed with Ep-GBM in Beijing Tiantan Hospital affiliated to Capital Medical University from May 2016 to Aug 2018. The patients’ clinical, pathological and radiological features were mainly summarized and evaluated. Results The mean age of patients with Ep-GBM was (43.2±19.7) years (range: 4-70 years). There was obvious enhancement and necrosis in the images of Ep-GBM and about 40.0% patients concurrent with cystic change. Besides, Ep-GBM showed high perfusion in the perfusion weighted imaging (PWI) and decreased NAA peak but increased Cho peak in the magnetic resonance spectroscopy (MRS). Molecular pathology detection results revealed that the positive rates of BRAF V600E and INI-l was 33.3%, 87.5%, respectively, while the median proliferation cell nuclear antigen (Ki-67) index was 50.0%. Both the frequency of IDH mutation and Chromosome 1p/19q deletion was 0%. The median progression-free survival (PFS) and overall survival (OS) of this cohort was 8.0 and 15.0 months. Conclusion Conclusions Ep-GBM, as a new glioblastoma subtype, harbors a poor prognosis. Gross total resection is the key of its treatment. TERT mutation is a molecular predictor of lethal clinical outcome.

关 键 词：上皮样胶质母细胞瘤 病理学 影像学 预后 

分 类 号：R739.4[医药卫生—肿瘤]