[目的]分析报道外阴上皮样肉瘤（epithelioid sarcoma, ES）诊断治疗过程，为临床提供罕见病例诊断治疗依据。[方法]通过分析患者的发病过程、治疗过程及病理学等检查，并对外阴ES的治疗方式、预后效果进行总结，真实地记录外阴ES的发生发展、治疗过程及预后。[结果]患者术中病理检查诊断为外阴ES，并给予扩大清除术，现患者无复发。[结论]外阴ES临床罕见，确诊时需要结合免疫组化检查，局部扩大清除术是其有效的治疗方法。%Objective Analyse and report the diagnosis and treatment process of vulvar epithelioid sarcoma, it provides a basis for rare case in clinic. [Methods] Through the analysis of the patient's il ness, treatment, and pathology examination, and the effect of the treatment of prognosis of vulvar epithelioid sarcoma of the summary, truly record the occurrence of vulvar epithelioid sarcoma development, treatment and prognosis. [Results] The intraoperative pathologic examination in patients diagnosed the vulvar epithelioid sarcoma, and it used the removal of expanding. Now the patient is not recurred. [Conclusion] Vulvar epithelioid sarcoma is clinical y rare, needs combined with immunohistochemical examination at the time of diagnosis, removal of local expansion is the effective method to treat, it is not exactly curative effect of chemotherapy and radiotherapy.
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