腺泡状软组织肉瘤的临床治疗分析

Diagnosis and treatment of alveolar soft tissue sarcomas

目的探讨腺泡状软组织肉瘤手术切除及辅助放、化疗的临床治疗效果。方法对1993～2006年间收治的腺泡状软组织肉瘤12例回顾性分析。男5例,女7例。年龄11～37岁。肿瘤直径平均5.5cm。结果12例手术后切除缘评价中,达到广泛切除缘者10例,边缘切除缘者2例,术后辅助放疗2例,全组均予辅助化疗。12例手术后复发3例,占25%。有7例发生转移,首诊时转移2例,其中肺转移瘤5例,脑转移瘤2例。转移率58.3%。转移瘤单纯化疗5例中PR3例,NC1例,PD1例,3例PR患者均采用MAI方案。随访时间10个月～10年6个月,12例中7例生存,5例死亡,经过Kaolan-Meire生存率计算,3年生存率81.2%,5年生存率52.8%。结论腺泡状软组织肉瘤外科治疗应以广泛切除术为基本原则,术后辅助放疗可以减少复发,辅助化疗是转移瘤的主要治疗方法。

Objective To investigate the clinical treatment of alveolar soft tissue sarcomas（ASTS）, Methods Clinical data of 12 patients with ASTS treated from 1993 to 2006 were retrospectively analyzed. Results Complete tumor resection was performed in 10 cases, The partial excision was performed in 2 cases. There were 2 cases treated with postoperative radiotherapy. All cases were treated with postoperative chemotherapy. Local recurrence occurred in 3 of 12 cases （25%）. Metastases developed in 7 cases, 5 in lung and 2 in brain （the rate of metastasis was 58.3%）. Seven cases were alive and 5 cases died. The 3 years survival rate was 81.2% and the 5 year survival rate was 52.8 %. Conclusions Complete resection is the most important factor for the treatment of ASTS. Postoperative adjuvant prophylatic radiotherapy might reduce local recurrence. Adjuvant chemotherapy is the major treatment for metastasis.