上皮样肉瘤的症状和治疗

Symptom and treatment of epithelioid sarcoma

目的 报道 5例手部上皮样肉瘤 (epithelioidsarcoma)并结合国内外 2 9篇文献介绍其临床表现 ,治疗方法及预后。方法 5例肿瘤均在手掌部 ,皮肤无异常 ,伴不适感或轻度压痛。 4例的受累指伸直受限 ,呈轻度屈曲状。肿瘤的长径为 1.0～ 5 .0cm ,质地硬韧 ,边界不清 ,底部固定。均行肿瘤边缘切除术。结果 病理诊断为上皮样肉瘤。 2例在术后 2个月内复发 ,1例作截肢术 ,1例拒绝手术。 3例术后随访 3～ 18个月 ,无复发。结论 上皮样肉瘤好发于青少年及手部 ,初起时无明显症状 ,为手部常见软组织恶性肿瘤之一。无特异性症状及体征 ,国内用肿瘤边缘切除术和扩大切除术 ,国外用根治术。诊断依靠病理结果 ,术后复发率高 ,预后不佳。

Objective To report 5 cases of epithelioid sarcoma in the hand,and analyze the clinical behavior, treatment methods and prognosis through review of the literatuer. Methods All the tumors occurred in the palm of the hand.No skin abnormality was seen. There was discomfort and slight tenderness. The 4 involved fingers had difficulty in extension, with slight flexion deformity. The tumor was 1.0-1.5 cm in diameter, hard and tenacious in nature, with unclear border and fixed. All of the 5 cases was treated by marginal resection. Results The pathological diagnosis was epithelioid sarcoma. Two of the patients had a recurrence 2 months after the operation. One of them had amputation, while the other refused further operation. During the follow-up period from 3 to 18 months, there was no recurrence in the rest 3 cases.Conclusion Epithelioid sarcoma is one of the most common malignant soft tissue tumors of the hand in teenages. There are no specific symptoms and signs. Marginal resection and extensive resection have been reported domestically, while radical resection is reported abroad. Its diagnosis is based on pathological results. It has a high recurrence rate and bad prognosis.