上皮样胶质母细胞瘤4例临床病理分析

发布时间：2018-03-18 21:39

本文选题：脑肿瘤　切入点：胶质母细胞瘤　出处：《中国神经精神疾病杂志》2017年08期 　论文类型：期刊论文

【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。
[Abstract]:Objective to investigate the imaging and pathological features of epithelioid glioblastomae (E-GBM) and to improve the diagnostic accuracy of E-GBM. Methods the clinical data of 4 patients with E-GBM were analyzed retrospectively. The histomorphology and immunohistochemical phenotypic features of the rare glioblastoma subtype were summarized in combination with the review of the literature. Results the MRI of 4 young patients with E-GBM were all irregular cystic and solid occupying space. The histopathological features were epithelioid or rhabdomyoid tumor cells with flake distribution, lack of adhesion, eosinophilic cytoplasm, and mitosis. Immunohistochemical staining showed that 4 tumor cells were positive for Vimentin and S-100 in varying degrees, but only 1 case of GFAP was focal positive. The positive rate of BRAFFTVE1) could be as high as 75% of IDH-1 negative and no deletion of INI-1. There was no deletion of 1 p / 19 Q chromosome, 1 case died 6 months after operation and 1 case recurred at 3 months after operation. Conclusion the prognosis of E-GBM is poor. It has a close relationship with the pleomorphic xanthoastrocytoma, and its special population, imaging features and molecular genetic phenotype are the key points in the differentiation of the tumor.
【作者单位】：广东三九脑科医院病理科;暨南大学附属华侨医院病理科;中山大学附属第一医院病理科;
【分类号】：R739.4

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