上皮样胶质母细胞瘤4例临床病理分析  

Clinical and pathological analysis of epithelioid glioblastoma: a report of 4 cases

作　　者：李海南 刘敏婷 成丽娜 张振斌 杨丽 汪文胜 蒋光愉 李智[3] 

机构地区：[1]广东三九脑科医院病理科,广州510510 [2]暨南大学附属华侨医院病理科 [3]中山大学附属第一医院病理科

出　　处：《中国神经精神疾病杂志》2017年第8期458-463,共6页Chinese Journal of Nervous and Mental Diseases

摘　　要：目的探讨上皮样胶质母细胞瘤(epithelioid glioblastoma,E-GBM)的影像和病理学特征,提高E-GBM诊断准确性。方法收集4例E-GBM患者临床资料,回顾性分析病例的影像学、组织形态学及免疫组织化学表型特点。结合文献复习,总结该罕见胶质母细胞瘤亚型的临床病理特征。结果 E-GBM中青年多见,4例患者MRI均为不规则囊实性占位,增强后呈不规则斑片或明显环状强化。病理组织学均表现为上皮样或横纹肌样肿瘤细胞,呈片状分布,瘤细胞缺乏粘附性,胞浆嗜酸性,核分裂多见,并见大片地图样坏死。免疫组化显示4例肿瘤细胞Vimentin和S-100不同程度阳性,但GFAP仅1例灶性阳性。BRAF(VE1)阳性率可高达75%,IDH-1阴性、INI-1无缺失,且均无1 p/19 q染色体臂共缺失。术后随访2~6个月,1例患者术后6个月死亡,1例术后3个月复发,2例随访期间未见复发。结论 E-GBM预后较差,且与多形性黄色星形细胞瘤关系较为密切,其特殊的发病人群、影像学特征和分子遗传学表型是该肿瘤鉴别的关键要点。Objective To investigate the neuroimaging and pathological features of epithelioid glioblastoma（EGBM） to improve the diagnosis. Methods The clinical and pathological features of 4 E-GBM cases were analyzed retrospectively. Results E-GBMs occurred predominantly in young adults. MRI examination showed irregular solid-cystic lesion with heterogeneous or ring enhancement in 4 cases. Histological examination revealed uniform population of epithelioid or rhabdoid cells with prominent nucleoli and mitotic activity as well as geographic necrosis..Immunohistochemical staining showed various positive signals of Vimentin and S-100 protein in 4 cases, positive signal of BRAF（VE1） in 3 cases and focal positive signal of GFAP in 1 case. However, IDH-1 was negative and 1p/19 q codeletion was lack. All patients were followed-up for 2-6 months. One patient had tumor recurrence 3 months and one patient died of disease 6 months after surgical excision. Conclusion E-GBM has a poor outcome and is closely associated with pleomorphic xanthoastrocytoma. MRI and epithelioid histological features are very important for the differential diagnosis.

关 键 词：脑肿瘤 胶质母细胞瘤 病理学 诊断 鉴别 

分 类 号：R739.4[医药卫生—肿瘤]