恶性胃肠神经外胚层肿瘤病理观察  

Maliganant gastrointestinal neuroectodermal tumor: a case report and review of literature

作　　者：雍翔 谷从友[1] 武世伍[1] 陶仪声[1] 

机构地区：[1]蚌埠医学院第一附属医院病理科,安徽蚌埠233000

出　　处：《诊断病理学杂志》2015年第6期347-350,353,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨恶性胃肠神经外胚层肿瘤(GNET)的临床病理学特点。方法对1例GNET进行光镜观察、免疫组化标记及荧光原位杂交(FISH)检测,并复习相关文献。结果镜下肿瘤位于胃壁黏膜下层及肌壁间,瘤细胞呈圆形、卵圆形至梭形,片状或巢状分布。局部瘤细胞排列成假腺样和假乳头样,瘤细胞中等大小,胞质丰富、淡嗜酸;核呈空泡状,部分瘤细胞有小核仁及核内包涵体,核分裂象4个/10HPF。间质内可见破骨细胞样巨细胞,散在分布。免疫组化:瘤细胞vimentin和S-100(+),HMB-45、melan-A和CD117等均(-);破骨样多核巨细胞CD68(+)。FISH信号异常(如1F1G1R等)的细胞比例为71%(>阈值15%),EWSR1基因断裂。结论恶性胃肠神经外胚层肿瘤可能为一独特临床病理单元,应与原发于胃肠道的透明细胞肉瘤、恶性黑色素瘤等相鉴别。Objective To investigate the clinicopathological characteristics of the maliganant gastrointestinal neuroectodermal tumor （GNET）.Methods One case of GNET was studied by light microscopy,immunohistochemistry,fluorescence in situ hybrdisation （FISH） and review of the literature.Results Microscopically,the tumor was located in submucosa and muscularis propria of the stomach.Tumor was characterized by solid uniform neoplastic population composed of diffuse sheets or nests of medium-size round or oval-to-spindle tumor cells.It focally displayed pseudoalveolar and pseudopapillary architecture.Tumor cells displayed a predominantly epithelioid appearance and contained round or oval nuclei surrounded by variable amounts of eosinophilic cytoplasm.The nuclei showed vesicular chromatin with peripheral margination,generally indistinct to small nucleoli,occasional prominent nucleoli,and intranuclear cytoplasmic inclusion.Mitotic activity was 4 mitoses per 10 HPF.Scattered distributed osteoclast-type giant cells were noted in the tumor.The most neoplasm cells were strong and diffuse nuclear and cytoplasmic positivity for S-100 protein,vimentin,but HMB-45,Melan-A,CD117 and other markers were negative.FISH studies showed EWSR1 was split-apart signals in the neoplastic cells.Conclusion GNET may be a new neoplastic entity and shoule be differentiated from clear cell sarcoma,malignant melanoma and so on.

关 键 词：胃肠肿瘤 神经外胚层肿瘤 免疫组化 荧光原位杂交 

分 类 号：R735[医药卫生—肿瘤]