15例腺泡状软组织肉瘤临床特点及预后分析

摘要

背景与目的:腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS)是一种罕见的恶性软组织肿瘤,生长缓慢,无典型临床症状,常伴有远处转移(肺转移,脑转移及骨转移等).本文旨在研究腺泡状软组织肉瘤的临床特点,评估其预后因素.方法:回顾性研究了2003年1月-2008年12月就诊于北京大学人民医院骨与软组织肿瘤治疗中心的15例腺泡状软组织肉瘤患者的临床资料,并随访了他们的预后情况.15例患者中,女性9例,男性6例;小于或等于30岁者8例,大于30岁7例:肿瘤大于5 cm者11例,小于5 cm者4例:原发于软组织12例,原发于骨骼3例;未见其他部位转移6例,肺转移6例,骨转移3例,脑转移2例:术后局部复发2例;术后9例接受放疗,6例未接受放疗;术后接受化疗11例,未接受化疗4例,化疗方案MAID:多柔比星,氮唏米胺及异环磷酰胺.结果:患者预后情况较差,其中无瘤生存6例,带瘤生存4例,死亡5例,中位生存时间为42.0个月,5年生存率为41.7%.伴有远处转移(肺转移、脑转移及骨转移)的患者5年生存率远低于整体生存率.患者个人性别、年龄对预后无显著性差异.肿瘤大小、部位及有无远处转移是影响预后的重要因素.结论:腺泡状软组织肉瘤是高度恶性软组织肿瘤.尽早评估病情,制定合适的治疗方案,原发病灶采取广泛切除,以降低复发及转移风险.但由于本组数据较少,放化疗是否有效还需进一步研究.%Background and purpose: Alveolar soft part sarcoma (ASPS) is a rare malignant neoplasm characterized by slow growth and indolent behavior, and most of the patients had metastases in diagnoses. This study was to define clinical features and assess prognostic factors of ASTS. Methods: Between January 2003 and December 2008, there were 15 patients with ASTS who received treatment and were followed up in the Department of Orthopedic Ontology of Peking University People's Hospital. We retrospectively analyzed the clinical factors of the disease related to prognosis: gender, age, tumor size, primary tumor site, metastatic sites, local recurrence, the roles of postoperative radiotherapy and postoperative chemotherapy. Resnlts: At a follow-up from 7 to 76 months, only 2 patients had local recurrences after excision, but there was a high incidence of pulmonary metastases. Only 6 patients achieved disease free survival, 5 patients died of metastases or other complications. The five-year overall survival rates was 41.7%, and the median survival time was 42.0 months. Univariate analysis and Cox regression analysis revealed that tumor size, tumor primary site and metastases at diagnoses were prognostic factors, but not gender and age, Conclusion: Alveolar soft part sarcoma is a high grade malignant soft tissue neoplasm with frequent metastases to lung, bone or brain. Tumor size, primary tumor site and metastases at diagnoses are some of the prognostic factors. Chemotherapy or radiotherapy after surgery have no benefit to the survival in our analysis. Further prospective studies are needed to clarify the best strategy for the treatment of ASPS.