胃原发胚胎性横纹肌肉瘤临床诊断学特征及文献复习

Clinical diagnostic features and literature review of primary gastric embryonal rhabdomyosarcoma

目的探讨胃原发性胚胎性横纹肌肉瘤的临床诊断学特征。方法回顾性分析1例临床表现为成人原发于胃的胚胎性横纹肌肉瘤患者的临床资料,并进行相关文献复习。结果患者表现为腹痛、腹部肿物等,胃镜显示胃贲门、胃底、胃体占位,贫血胃,胃窦息肉。胃镜病理结果提示胚胎性横纹肌肉瘤,镜下肿瘤细胞弥漫分布,排列呈片状、巢状,瘤细胞较小,呈圆形,核深染,居中或偏位,胞质少,核分裂易见。免疫组化Myogenin+,Desmin+。结论成人原发于胃的胚胎性横纹肌肉瘤是一罕见的高度恶性软组织肿瘤,需要结合临床影像学、病理学、免疫学等多种诊断方法,可作出正确诊断。

Objective To explore the clinical diagnostic features of embryonal rhabdosarcoma （ERMS）.Methods One patient with ERMS caused by tumor was diagnosed and the clinical data were retrospectively analyzed. The relevant literatures were reviewed. Results The patient presented with bellyache,palpable abdominal mass. Gastroscopy revealed space occupying lesion of cecus minor ventriculi, gastric fundus and gastric body,Biermer-Ehrlich anemia and polyps of gastric antrum.Pathologic diagnosis was ERMS.Microscopically,tumor cells shaped aerie and sheet.The tumor cells were round and small,with less cytoplasm.The nucleus was located in the central or the partial position,with apparent hyperchromatism and fission. Immunohistochemically, it was positive for Myogenin and Desmin. Conclusions ERMS in stomach of adult is a high grade malignant soft tissue neoplasm,and it is rarely to be found.Careful analysis of the clinicopathological features and immunohistochemisitry will be helpful to make accurate diagnosis.