近端型上皮样肉瘤临床病理研究

目的 探讨近端型上皮样肉瘤(PES)的临床病理特点、诊断及鉴别诊断依据.方法 收集5例PES患者资料,免疫组织化学EnVision法染色.第一抗体选用细胞角蛋白(CK)、波形蛋白、上皮细胞膜抗原(EMA)、CD34、β-catenin、S-100蛋白、平滑肌肌动蛋白(SMA)、肌调节蛋白(MyoD1)、结蛋白、HMB45、CK7及CK20,观察和分析其临床病理学形态及免疫表型特征.结果 5例PES中女1例,男4例;发病年龄19-46岁,发生部位分别为:会阴部2例,下腹部、髂前上棘和臀部各1例,均表现为进行性增大的无痛性单发肿块.光镜下肿瘤细胞呈结节状排列,浸润性生长,瘤细胞大部分由相对独特的上皮样细胞组成,胞质丰富,嗜酸性;核卵圆形,肿瘤中心常见坏死.免疫组织化学染色示5例瘤细胞均表达波形蛋白,4例表达CK、EMA,3例表达β-catenin、CD34,1例表达S-100蛋白;而SMA、MyoD1、结蛋白、HMB-45、CK7及CK20均阴性.结论 根据PES组织学和病理学特征,结合免疫组织化学染色结果可以做出明确诊断.

Clinicopathological study of 5 cases of proximal-type epithelioid sarcoma

Objective To investigate the clinicopathological characteristics,diagnosis and differential diagnoses of proximal-type epithelioid sarcoma (PES). Methods Five cases of PES were retrieved from pathology Fries. Clinical,pathologic and immunohistochemical features of the tumors were reviewed. Results One patient was female and 4 were male. Ages of the patients ranged from 19 to 46 years. The sites of the tumor involvement were vulvar (2 cases),hypogastric zone ( 1 case),anterosuperior iliac spine (1 case) and buttock (1 case). Clinically,the tumor masses were painless and progressive solitary nodules. Microscopically,the tumor cell growth was infiltrative in nature,nodular in appearnce with degenerative and necrotic ceils at the central areas. The tumors consisted of relatively uniform epithelioid cells with round or oval nuclei and eosinophilic cytoplasm,Immunohistochemically,the tumor cells were positive for vimentin (5/5),CK (4/5),EMA (4/5),β-catenin (3/5),CD34 (3/5),and S-100 protein (1/5),but were negative for SMA,MyoD1,Desmin,HMB-45,CK7 and CK20. Conclusion Definitive diagnosis of PES relies on its histopathological characteristics in conjunction with appropriate immunohistochemical findings.