胃肠道恶性神经外胚层肿瘤临床病理分析合并文献复习  

Clinicopathologic Analysis of Malignant Gastrointestinal Neuroectodermal Tumor and Review of the Literature

作　　者：何同梅[1] 张和军[1] 陈雪燕[1] 陈丽芳[1] 李鑫静[1] 何时[1] 陈燕坪[1] 许春伟[1] 陈刚[1] 

机构地区：[1]福建省肿瘤医院,350014

出　　处：《实用癌症杂志》2017年第7期1113-1115,1125,共4页The Practical Journal of Cancer

基　　金：国家临床重点专科建设项目(编号:2013);福建省卫生厅青年创新课题(编号:2014-1-15)

摘　　要：目的探讨胃肠道恶性神经外胚层肿瘤的临床病理学特点、免疫表型、分子遗传学改变、诊断及鉴别诊断要点。方法回顾性分析胃肠道恶性神经外胚层肿瘤的临床资料、光镜观察、免疫组化标记及荧光原位杂交检测,并结合相关文献分析。结果肿瘤位于升结肠,浸润性生长,侵犯肠壁全层。肿瘤组织弥漫成片状或巢状生长,局部区域呈腺泡状及流水样排列。肿瘤细胞呈上皮样,局部呈梭形,胞质嗜酸性或透明,细胞核圆形或卵圆形,染色质稀疏,有小核仁。核分裂象6个/10HPF。免疫组化:Vimentin(+),SOX10(+),S-100(+),Syn灶性(+),CD56(+),Fli1(+),Ki-67增殖指数20%,荧光原位杂交检测显示EWSR1基因重排。结论胃肠道恶性神经外胚层肿瘤非常罕见,具有高度侵袭性,应与其它胃肠道原始上皮及梭形细胞肿瘤相区别。联合应用免疫表型、超微结构和分子学分析,有助于识别该肿瘤并与其他类似病变鉴别。Objective To explore the characteristics of clinicopathology,immunophenotype,molecular alteration,diagnosis, differential diagnosis of malignant gastrointestinal neuroectodermal tumor （MGNET）. Methods A retrospective analysis of clinical data,light micmscopy,immunohistochemistry, fluorescence in situ hybridization of 1 case of MGNET was conducted, and review of the 1 iterature were conducted. Results Histologically, the neoplasm involved the full thickness of the ascending colon. Tumor cells, mainly displayed epithelioid or spindle appearance with oval or round nuclei, arranged in strand, nested, and solid pattern. Nuclear division was easy to see. Immunohistochemically, the tumor cells were strongly positive for Vimentin, SOX10, S100, CD56, Flil, and partly positive of Syn, The Ki67 proliferation index was about 20%. Fluorescence in situ hybridization assay revealed EWSR1 gene rearrangement. Conclusion MGNET is very rare and highly invasive, should be distinguished with other gastrointestinal primitive epithelial and spindle cell tumors. Analysis of combined application of immunophenotype, uhrastructure and molecular helps to identify the tumor and other similar disorders.

关 键 词：胃肠道恶性神经外胚层肿瘤 鉴别诊断 免疫组化 荧光原位杂交 

分 类 号：R735.2[医药卫生—肿瘤]