上皮样肉瘤的临床病理研究

Clinicopathological study of epithelioid sarcoma

目的探讨上皮样肉瘤(ES)的临床、病理学特征及鉴别诊断要点。方法收集17例上皮样肉瘤患者资料,观察和分析其临床和组织病理学特征,通过免疫组化方法分析其细胞角蛋白(CK)、上皮膜抗原(EMA)、波形蛋白(Vimentin)、S-100蛋白、CD34及平滑肌肌动蛋白(SMA)的表达。结果 17例患者中,男性13例,女性4例,男性多见。平均年龄35.6岁。远端型6例,近心型11例。两型病理形态不同,但免疫组化表达一致,均表达CK、EMA、Vimentin和CD34。初次手术后1年、3年复发及转移率分别为50.0%(8/16)和87.5%(14/16)。结论 ES是一种极易局部复发和远处转移的恶性肿瘤,免疫组化有助于诊断及鉴别诊断。根治性手术是首选治疗方案,ES易发生淋巴结转移,区域淋巴结清扫是必要的。

Objective To investigate clinical and pathological characteristics of epithelioid sarcoma（ES）and summarize key points of ES differential diagnosis.Methods 17 ES cases were collected.Clinical and pathological characteristics of all the cases were analyzed and immunohistochemistry（IHC）analysis was applied to investigate expressions of cytokeratin（CK）,epithelial membrane antigen（EMA）,Vimentin,S-100,CD34 and smooth muscle actin（SMA）.Results ES was more common in male than in female（13 cases vs 4 cases）and the average age at diagnosis was 35.6 years.Among the 17 ES cases,six of them were distal type and eleven of them were proximal type.The pathological morphology was different between distal and proximal types,however the IHC expression pattern was the same,with CK,EMA Vimentin and CD34 expressed in all the cases.One-year and three-year ES recurrence/metastasis rates were 50%（8/16）and 87.5%（14/16）,respectively.Conclusions ES is a malignant tumor type,which is easily to develop local recurrence and distant metastasis.IHC analysis is useful in ES diagnosis and differential diagnosis.Radial resection is the first choice of treatment.ES is easily to develop lymph node metastasis;therefore regional lymph node dissection is necessary.