近端型上皮样肉瘤1例临床病理分析

Clinical and pathological analysis on a case of proximal-type epithelioid sarcoma

目的研究近端型上皮样肉瘤(PES)的临床病理学特征、免疫表型、诊断和鉴别诊断、治疗及预后。方法回顾性分析1例PES患者的临床病理学特点、免疫表型并复习相关文献。结果肿瘤位于头部,临床表现无特异性,光镜下由多角形上皮样及梭形细胞组成多个结节,部分结节中央见坏死,瘤细胞胞浆嗜酸性,免疫组化:广谱细胞角蛋白PCK抗体(pan-CK)、上皮膜抗原(EMA)、Vimentin、CD34及CA125均阳性。结论 PES是一种发病率低的软组织肿瘤,尤其是头部,其诊断依赖于镜下形态及免疫表型。

[Objective]To study the clinicopathologic features,immunophenotype,diagnosis and differentiation,treatment and prognosis of proximal-type epithelioid sarcoma（PES）.[Methods]Clinicopathological features and immunophenotype of one case of proximal-type epithelioid sarcoma were retrospectively analyzed,and related literatures were reviewed.[Results]This tumor occurred in head,and no special clinical symptoms was found in this tumor.Histologically observation showed that tumor cells consisted of polygonal epithelioid cells and spindle cells,all cells were put in multinodular mass with central necrosis.The cells had eosinophilic cytoplasm,immunophenotype：pan-cytokeratin（CK）,epithelial membrane antigen（EMA）,vimentin,CD34 and CA125 staining were positive.[Conclusion]PES is a relatively rare soft-tissue sarcoma,especially in head,and it could be diagnosed by pathological features and immunophenotype.