上皮样肉瘤的诊治分析

Diagnosis and treatment of epithelioid sarcoma

目的分析上皮样肉瘤（ES）的临床特点、治疗模式及预后。方法回顾性分析1995年3月至2008年12月间天津医科大学附属肿瘤医院收治的13例ES患者的临床资料、治疗和随访情况。其中男性10例，女性3例，平均年龄41．5岁。肿瘤发生于上肢6例，下肢4例，腰背部3例。全组除1例患者因远处血行转移行姑息化疗外，其余12例均行手术治疗。术后单纯放疗4例，单纯化疗5例，放疗＋化疗1例。采用SPSS16．0统计软件包，以Kaplan—Meier法进行生存分析。结果13例ES患者中，12例为单发肿物，肿物平均直径为（6．07±1．34）cm；有6例（46．2％）患者发现区域淋巴结转移。10例随访患者中，5例（50．0％）出现肿瘤局部复发，3例（30．0％）出现远处转移，其中以肺转移最常见；4例患者于术后2年内死亡。全组患者术后1、2、5、10年生存率分别为72．7％、54．5％、27．3％和9．1％，中位生存时间为27个月。结论ES临床罕见，易发生局部复发、淋巴结扩散和（或）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ES的主要治疗方式。

Objective To analyze the clinical features, diagnosis, treatment, and prognosis of epithelioid sarcoma（ES）. Methods The clinical data of 13 cases with epithelioid sarcoma in the Tianjin Medical University Cancer Institute and Hospital from March 1995 to December 2009 were collected and analyzed. There were 10 males and 3 females in the group, with an average age of 41.5 yeas （ range： 13 to 68 years）. Nine patients had classic ES and 4 had proximal-type ES. Surgery was the mainstay of treatment. After the operation, four patients received radiotherapy, five received chemotherapy, and one received chemoradiotherapy. Results Of the 13 cases, only 1 had multi-locus lesion. The average tumor size was （6.07± 1.34） cm. The lymph node involvement was found in 46.2% of the patients. Local and distant failure occurred in 50% and 30% patients, respectively. The most common site for dissemination was the lung. Four cases died within 3 years after initial operation. The 1-, 2-, 5-, 10-year overall survival rates of the 11 cases were 72.7% , 54.5% , 27.3% and 9.1% , respectively, with a median survival time of 27 months. Conclusions Epithelioid sarcoma is a rare disease. The prognosis for patients with epithelioid sarcoma is poor because of a high propensity for local recurrence, lymph node metastases, and/or distant metastases. The definite diagnosis depends mainly on the pathologic examination. Wide surgical excision is the mainstay treatment, and radiation and chemotherapy have been used occasionally as adjuvant therapy but have had limited success.