上皮样肉瘤8例报道及文献复习

    Epithelioid sarcoma:a report of 8 cases and review of literature

    • 摘要: 目的:探讨上皮样肉瘤(ES)的临床病理学特征及免疫表型。方法:对8例ES标本,通过常规组织学和免疫组织化学S-P法,进行光镜观察,并复习有关文献。结果:本组男5例,女3例;平均年龄42.9岁。3例为远端型,均位于前臂;镜下可见不规则肉芽肿样结节,中心有坏死,周边为上皮样瘤细胞;5例为近端型,见于会阴、颈部和胸部,上皮样和横纹肌样瘤细胞呈片状或弥漫分布,异型性较明显,核分裂较多。免疫表型细胞角昼白(CK)(8/8)、波型昼白(vimentin)(8/8)、上皮膜抗原(EMA)(6/8)、CD99(5/5)、神经元特异性烯醇酶(NSE)(4/5)、CA125(4/5),而其他标记均为阴性。结论:ES可分为远端型和近端型,但两者的免疫表型相似,均表达CK、Vimentin和EMA,有助于与其他具有上皮样细胞形态的软组织肿瘤相鉴别。

       

      Abstract: Objective:To explore the clinicopathological characteristics and immunophonetype of the epithelioid sarcoma(ES). Methods:Eight cases of ES were observed microscopically by using routine methods and immunohistochemical techniques (S-P method),and the related literature were reviewed. Results:Eight cases of ES included 5 males and 3 females with mean age of 42. 9 years. Three cases of distal type ES developed in the forearms and characterized by the formation of irregular nodules with central necrosis which were surrounded by epithelioid cells. While in the other 5 cases of proximal type ES occurred in the vulva, chest and neck, respectively. Epithelioid and rhabdoid cells formed large sheets or disturbed diffusely. The tumor cells showed marked atypia and more mitotic figures in this type. Most tumor cells expressed CK(8/8), vimentin(8/8),EMA(6/8),CD99(5/5),NSE(4/5),and CA125(4/5). While the other markers were almost negative. Conclusions:Epithelioid sarcoma could be divided into distal and proximal types based on the developed location of the tumors which were different histopathologically. The same immunophenotypes including positive expression for CK, EMA, vimentin, etc are helpful to the diagnosis and differential diagnosis of soft tissue neoplasms with epithelioid cells.

       

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