上皮样肉瘤5例病理学特征分析

Clinicaopathologici features of epithelioid sarcoma: an analysis of 5 cases

目的探讨上皮样肉瘤的临床病理学特征和鉴别诊断。方法回顾性分析5例上皮样肉瘤的临床表现、组织学特点和免疫组化,并结合文献探讨上皮样肉瘤的临床病理特点、诊断与鉴别诊断。结果 5例均为手术切除标本,其中2例为截肢标本。男性4例,女性1例,男性多见。平均年龄31.4岁。镜下观察肿瘤由梭形和上皮样的细胞组成,两种细胞在形态上有移行。免疫组化显示上皮和间叶组织双向分化,5例CK、EMA和vimentin均(+),desmin和HMB45均(-)。2例CD34(+),2例SMA(+)。结论上皮样肉瘤是一种较罕见恶性肿瘤,由于其临床表现无特异性,确诊需依赖于病理组织学和免疫组化染色,同时与形态学上同样具有上皮样或者横纹肌样细胞特点的其他疾病进行鉴别诊断。

Objective To suudy the clinicopathololgic features and differential diagnosis of epithelioid sarcoma （ES）. Methods The clinical manifestation, histological characteristics and immunophenotype were retrospectively analyzed in 5 cases of epithelioid sarcoma, with review of literature to explore the pathological features, diagnosis and differential diagnosis. Results 5 cases were surgically resected specimens, including 2 case of amputation ; 4 cases were male and 1 was female. The average age was 31.4 years. Microscopically, the tumor was composed of spindle and epithelioid cells ; two types of cells had transitional form. Immunohistochemicstry showed that epithelial and mesenchymal tissues had two-way differentiation： 5 cases expressed CK, EMA, and vimentin, but not desmin and HMB45. CD34 and SMA expression was found in 2 cases, respectively. Conclusion Epithelioid sarcoma is a rare malignant tumor, due to its lack of characteristic clinical manifestations, diagnosis depends on pathological histology and immunohistochemical staining. At the same time, the differential diagnosis should be made from other lesions with the similiar characteristics of epithelioid or striated muscle cells morphology.