成人胃原发胚胎性横纹肌肉瘤临床病理特征及文献复习  

Primary embryonal rhabdomyosarcoma of the stomach in adults: clinicopathological features and review of literature

作　　者：赵元 马强 兰秋霞 肖华亮 ZHAO Yuan;MA Qiang;LAN Qiu-xia;XIAO Hua-liang(Department of Pathology,Daping Hospital,Army Medical Universityy Chongqing 400042,China)

机构地区：[1]陆军军医大学大坪医院病理科,重庆400042

出　　处：《诊断病理学杂志》2022年第4期349-353,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的 探讨成人胃原发胚胎性横纹肌肉瘤的临床病理特征、免疫表型及鉴别诊断。方法 对陆军军医大学大坪医院2019年确诊的1例成人胃原发胚胎性横纹肌肉瘤进行临床病理分析,并复习相关文献。结果 患者男性,55岁,因黑便、进食后哽咽感就诊。胸腹部CT示胃底贲门部胃壁明显增厚,黏膜下并见软组织肿块影,大小约14 cm×6.1 cm。胃镜显示:食管下段近贲门可见新生物,延续至贲门下胃底,可见巨大球状隆起,表面大片状溃烂,附着厚苔,不平整。显微镜下显示肿瘤细胞于胃壁全层浸润性生长,黏液样基质中细胞稀疏和细胞密集区域混杂,细胞异型性明显,梭形、圆形至椭圆形,细胞核小,深染,核分裂象易见。免疫表型及阳性率:Desmin (20%+),MyoD1 (30%+),Myogenin (80%+)。FISH检测未见FOXO1易位。结论 成人原发于胃的胚胎性横纹肌肉瘤是较罕见的高度恶性软组织肿瘤,病理形态学及免疫表型均有一定特异性,需要与低分化癌、胃肠间质瘤、伴有横纹肌形态的软组织肿瘤等鉴别,术后予以靶向及免疫治疗,总体预后较差。Objective To investigate the clinicopathological features, immunophenotype and differential diagnosis of primary embryonic rhabdomyosarcoma of the stomach in adults. Methods A case of adult primary embryonal rhabdomyosarcoma of the stomach diagnosed in our hospital in 2019 was analyzed clinically and pathologically, and the relevant literature was reviewed. Results A 55-year-old male patient was presented with melena and choking-up after eating. CT of chest and abdomen showed obvious thickening of gastric wall in fundus and cardia, and soft tissue mass shadow under mucosa, about 14 cm × 6.1 cm in size. Gastroscopy showed that a neoplasm appeared near the cardia in the lower segment of the esophagus, extending to the gastric fundus under the cardia, with a huge spherical hump, large flab-like ulceration on the surface, thick moss attached and uneven. Microscopy showed that the tumor cells grew infiltratively in the whole layer of the gastric wall. In the myxoid matrix, the cells were sparse and the dense areas were mixed. The cell heteromorphism was obvious, fusiform, round to oval, the nucleus was small, deeply stained, with more mitotic figures. Immunophenotyping showed Desmin(20%+), MyoD1(30%+) and myogenin(80%+). No FOXO1 translocation was detected by FISH. Conclusion Adult primary embryonic rhabdomyosarcoma of the stomach is a relatively rare and highly malignant soft tissue tumor with certain specificity in pathomorphology and immunophenotype. It needs to be differentiated from poorly differentiated carcinoma, gastrointestinal stromal tumor, and other soft tissue tumor with rhabdomyoid morphology. After operation, targeted and immunotherapy are given, but the overall prognosis is poor.

关 键 词：胃 横纹肌肉瘤 临床病理诊断 鉴别诊断 

分 类 号：R735.2[医药卫生—肿瘤]