上皮样胶质母细胞瘤临床病理分析

目的 探讨上皮样胶质母细胞瘤(epithelioid glioblastoma,Ep-GBM)的临床病理特征、免疫表型及鉴别诊断.方法 收集2011年1月至2019年11月安徽医科大学附属巢湖医院及郑州市第一人民医院4例Ep-GBM临床病理及影像学资料、观察其病理学形态及免疫表型,并复习相关文献,探讨Ep-GBM的病理诊断和鉴别诊断要点.结果 4例Ep-GBM中,男3例,女1例.4例均发生于颞叶,临床表现为头痛伴恶心、呕吐,言语不清,感觉及语言障碍.影像学多提示囊实性肿物,可见出血、坏死及水肿.肿瘤切面灰白色、灰红色或灰黄色,无明显界限,质软或韧,可见囊性变及出血.镜检见肿瘤细胞以上皮样细胞及横纹肌样细胞为主,含有多少不等经典型胶质母细胞瘤成分.免疫组化:4例Vim与GFAP均(+),3例PCK(+)、INI-1(+);2例S-100、EGFR(+);1例Syn(弱+).随访4、8、9、13个月,4例均死亡.结论 Ep-GBM为罕见的高级别胶质瘤,容易沿脑脊膜播散,其确诊需结合临床病理特征进行综合分析.

Clinicopathologic study of epithelioid glioblastoma

Objective To investigate the clinicopathological characteristics, immunophenotypes and differential diagnosis of epithelial glioblastoma (Ep-GBM).Methods Fifteen cases of glioblastoma in Chaohu Hospital of Anhui Medical University and ZhengzhouFirst People''s Hospital from January 2011 to November 2019 were collected. The clinicopathological and imaging date of 4 cases of Ep-GBM were analyzed, the pathological morphology and immunophenotype were observed, and the relevant literature was reviewed to explore the key points of Ep-GBM pathological diagnosis and differential diagnosis.Results There were 4 patients with Ep-GBM, including 3 males and 1 female. The four cases occurred in the temporal lobe. The clinical manifestations were headache with nausea, vomiting, speech confusion, sensory and language disorders. Imaging findings suggested that cystic solid mass, visible bleeding, necrosis andedema. The cut surface of the tumor was grayish white, grayish red or grayish yellow, without obvious boundary, soft or tough, cystic degeneration and bleeding can be seen. Microscopic examination showed that the most tumor cells were epithelial cells and rhabdomyoidcells, containing some classical type of glioblastoma components. Immunohistochemically, all patients expressed Vimentin and GFAP,3 patients were CK, INI-1 positive, 2 patients were S-100and EGFR positive, 1 patient was Syn weak positive. The 4 cases received follow-up for 4, 8, 9 and 13 months. All four patients died.Conclusion Ep-GBM is a rare high grade glioma, which is easily disseminatedalong the cerebrospinal membrane, and its diagnosis needs to be combined with clinicopathological features for comprehensive analysis.