消化道原发性未分化多形性肉瘤2例临床病理观察

Primary undifferentiated pleomorphic sarcoma of digestive tract:a clinicopathological analysis of two cases

目的探讨消化道原发性未分化多形性肉瘤(UPS)的临床病理学特征。方法回顾性分析2例消化道UPS临床资料,行病理组织学检查及免疫组化观察,结合相关文献进行复习。结果 2例患者均为女性,年龄分别为45和60岁。临床主要表现为消化道肿块并伴有梗阻症状,遂行手术切除。镜下肿瘤细胞异型增生,排列呈旋涡状或席纹状,局灶可见凝固性坏死;细胞形态各异,呈多角形、梭形、类圆形等,并可见多核瘤巨细胞;肿瘤细胞胞质丰富,胞核大、深染,核仁明显,核分裂易见;间质局灶黏液样变性,少许中性粒细胞及淋巴细胞浸润。瘤细胞vimentin、CD68、AACT及lysozyme(+),CD117、Dog-1、CD34、SMA、desmin、S-100、HMB45、CD99、bcl-2、p16、CDK4、MDM2、EMA、广谱CK及ALK均(-),Ki-67阳性指数均>70%。结论消化道UPS是一种罕见的恶性肿瘤,组织起源目前尚不清楚,其诊断依赖于病理组织学检查及免疫组化。

Objective To investigate clinicopathological features of primary undifferentiated pleomorphic sarcoma （UPS） in the digestive tract.Methods With review of related literatures,the clinicopathologic data were retrospectively analyzed with characteristics of morphology and immunohistochemisty,observed in 2 patients with undifferentiated pleomorphic sarcoma of the digestive tract.Results Two female patients,aged 45 and 60 years,who presented with a gastrointestinal mass and obstruction symptoms,were performed with surgical resection.Microscopically,the tumor was mainly composed of dysplasia cells arranged in spiral or storiform patterns with focal coagulative necrosis.Tumor cells were mainly polygonal,spindle or round,with abundant cytoplasm and large nuclei,prominent central nucleolus,and many mitotic figures.Multinucleated and anaplastic cells were present.The stroma was focally myxiod with a mild neutrophilic and lymphocytic infiltration.Immunohistochemically,the tumor cells were positive for vimentin,CD68 and AACT,but negative for other markers,including lysozyme,CD117,Dog-1,CD34,SMA,desmin,S-100,HMB-45,CD99,Bcl-2,P16,CDK4,MDM2,EMA,panCK and ALK.Ki-67 index was more than 70％.Conclusion UPS is a rare malignant tumor in the digestive tract.Its histological origin is unclear,and the diagnosis depends on pathological analysis and immunohistochemical detection.