上皮样细胞肉瘤是一种软组织肉瘤,由于其生长缓慢,组织病理又无特异性,所以常在疾病的早期发生误诊。本文报告一例,以提请临床和病理工作者注意。患者男性,22岁。因右手2、3、4指外伤,而行清创和截肢术。17个月后,沿外科皮肤疤痕长一结节经切除后,病理报告为变性和反应性病变。其后患处皮疹三次复发,病理分别报告为结节性筋膜炎和结节性腱鞘炎。经会诊,最后确诊为上皮样细胞肉瘤,合并右上、下肺叶转移。经过截肢、肺叶切除及系统化疗,患者恢复良好出院,随访一年半疾病未复发。 Epithelioid cell sarcoma is a kind of soft tissue sarcoma. Due to its slow growth and no specificity in histopathology, it is often misdiagnosed in the early stage of the disease. This article reports an example to draw the attention of clinical and pathologists. The patient is male, 22 years old. Debridement and amputation were performed because the right hand 2, 3, and 4 were traumatic. After 17 months, along the length of the scar of the surgical skin, the pathology was reported as degeneration and reactive lesions. Afterwards, there were three recurrences of skin rash at the affected area. Histopathology was reported as nodular fasciitis and nodular tenosynovitis. After consultation, the final diagnosis was an epithelioid cell sarcoma with a combination of right upper and lower lobe metastases. After amputation, lobectomy, and systemic chemotherapy, the patient recovered well and was discharged after a year and a half without follow-up.