小肠平滑肌肉瘤临床病理观察

Clinicopathological features of small intestinal leiomyosarcoma

目的探讨肠道平滑肌肉瘤(LMS)的临床病理特征、免疫表型、鉴别诊断及预后。方法分析1例小肠LMS的临床病理学特征并复习相关文献。结果患者女性,53岁,因脐周疼痛1月余,加重1天入院。腹部CT示左腹空肠呈同心圆改变,肠壁增厚。患者行腹腔镜下腹腔空肠肿瘤切除术。术中见肠套叠位于左上腹空肠上段,套叠肠管复位后,可及肠腔内有一3 cm×5 cm大小肿块。镜下观察肿瘤组织由梭形细胞构成,胞质嗜酸性,核钝圆或呈雪茄样。细胞异型性明显,核分裂多见,局灶可见坏死。免疫组化:肿瘤细胞vimentin、SMA、desmin、H-caldesmon和SDHB(+)。CD117、Dog-1、CD31、CD34和melan-A均(-)。基因检测该肿瘤未发现KIT及PDGFRA基因突变。结论胃肠道LMS是一种非常罕见的肿瘤,明确诊断LMS需要综合组织学检查、免疫组化及基因分析。

Objective To investigate the clinicopathological features,immunophenotype,differential diagnosis and prognosis of intestinal leiomyosarcoma(LMS).Methods The clinicopathological features of 1 case of small intestine LMS were analyzed and the relevant literatures were reviewed.Results A 53-year-old female patient was admitted to the hospital due to periumbilical pain for more than 1 month and aggravation for 1 day.Abdominal CT showed concentric changes in the left jejunum and thickened intestinal wall.Laparoscopic resection of jejunal tumor was performed.The intussusception was located in the upper segment of the left upper abdominal jejunum.After reduction,a 3×5 cm mass was found in the intestinal cavity.Microscopically,the tumor was composed of spindle-shaped cells,with round or"cigarshaped"nuclei embedded eosinophilic cytoplasm.The nuclear atypia was obvious and mitosis was commonly noted.Necrosis was visible in the focal area.Immunohistochemistry showed that tumor cells were positive for vimentin,SMA,desmin,h-caldesmon and SDHB.CD117,DOG-1,CD31,CD34 and melanin-A were all negative.In addition,the tumor was lack of KIT and PDGFRA mutations.Conclusion Gastrointestinal LMS is a very rare tumor.Comprehensive histological examination,immunohistochemical examination and genetic analysis are necessary for the diagnosis of gastrointestinal LMS.