近端型上皮样肉瘤的诊治分析

Diagnosis and treatment of proximal - type epithelioid sarcomas

目的研究近端型上皮样肉瘤的临床特点、治疗方法及愈后分析，，方法回顾性分析2000年1月-2013年5月北京大学第一医院收治的7例近端型上皮样肉瘤病例，对其临床特点、病理学表现、诊治过程及预后进行分析。结果7例近端型上皮样肉瘤患者临床表现为发生于头颈及躯干部缓慢生长的皮下无痛性结节，均接受手术治疗，术后大部分（5／7）患者接受放射治疗，7例患者的1年、3年及5年生存率分别为100％、80％和60％。结论近端型上皮样肉瘤临床罕见，易局部复发、淋巴结转移及远处转移，患者预后不佳。明确诊断需病理学检查，手术切除仍是其主要治疗方式。

Objective To analyze the clinical features, treatment, and prognosis of proximal - type epi- thelioid sarcoma（PES）. Methods The clinical data of 7 cases with PES in the First Hospital of Peking Universi- ty from January 2000 to May 2013 were collected and analyzed, including clinical features, pathology, diagnosis, treatment and prognosis. Results All 7 patients with clinical manifestations of slowly growing painless subcutane- ous nodules in the head, neck and trunk received extensive resection surgery. The majority （ 5/7 ） of patients re- ceived radiotherapy postoperatively, and the survival rate of 1 - year,3 - year and 5 - year in our whole group was 100% ,80% and 60 %. Conclusion The prognosis of proximal - type epithelioid sarcoma is poor because of its high risk of local recurrence, node metastasis, and distant metastasis. PES is rare clinically and the pathological diagnosis is required. The early extensive resection surgery is still the main treatment of PES.