11例上皮样肉瘤临床治疗分析

CLINICAL RESEARCH ON 11 CASES OF EPITHELIOID SARCOMA

目的探讨上皮样肉瘤(epithelioid sarcoma,ES)临床特点、诊断及治疗方法,提高此病的诊疗水平。方法2000年1月～2006年9月,收治11例ES患者。男7例,女4例;年龄14～41岁。初发年龄9～41岁,平均27.7岁。上肢7例,下肢3例,腹壁1例。10例有复发史。肿瘤<2cm7例,2～5cm1例,>5cm3例。1例于外院行局部切除手术;7例行扩大切除术,切除肿瘤及周边3cm以上正常组织;3例行根治手术,截肢或截指。术后均行正规放、化疗治疗。结果患者术后伤口均期愈合,无并发症发生。皮瓣成活,植骨骨性融合。11例获随访5～54个月,平均23.2个月。8例术后2～20个月复发,平均8.9个月,复发率为73%。其中3例行截肢手术;4例术后6～24个月腋窝淋巴结转移,1例术后10个月肺部转移,均未再手术治疗。4例术后6～14个月全身衰竭死亡。结论对于ES,早期诊断可选择保肢的手术方式,术后予以辅助化、放疗是首选治疗方案。

Objective To investigate the clinical features, diagnosis, and treatment of patients with localized epithelioid sarcoma （ES）. Methods From January 2000 to September 2006, 11 patients with ES were treated. There were 7 males and 4 females aged 14-41 years. The patients＇ ages at the initial onset were 9-41 years, averaged 27. 7 years. The ES was located in the upper extremity in 7 patients,lower extremity in 3, and abdomen in 1. Among the patients, 10 had a recurrence. Tumor 〈2 cm was seen in 7 patients, 2-5 cm in 1, and 〉 5 cm in 3. One patient underwent an operation of local resection at another hospital. Seven patients underwent an expanding resection surgery, and the tumors with the surrounding normal tissues 3 cm above were removed. Three patients underwent a radical surgery, including extremity amputation or finger amputation. All the patients underwent routine radiotherapy and chemotherapy after operation. Results All the wounds had a healing at the first intention without complications. All the flaps survived and the grafted bone had a fusion. Among the 11 patients followed up for 5-54 months averaged 23.2 months, 8 had a recurrence 2-20 months （average, 8.9 months） after operation, with a recurrence rate of 73%. And among the patients, 3 had a further radical surgery of extremity amputation. Four patients had a metastasis in the axillary lymph nodes 6-24 months after operation, and 1 patient had a lung metastasis 10 months after operation. They did not have a further surgical treatment. Four patients died of systemic failure 6 14 months after operation. Conclusion An early expanding resection surgery combined with postoperative chemotherapy and radiotherapy is the therapy of choice for treating ES.