摘要目的 探讨腺泡状软组织肉瘤的临床特点与外科治疗的预后相关因素.方法 回顾性分析1982年1月至2010年10月期间资料完整的腺泡状软组织肉瘤29例,男18例,女11例;年龄9～58岁,平均24.4岁,中位年龄21岁.最终接受手术治疗的26例纳入研究,软组织为首发23例,骨首发3例.临床表现主要为局部的无痛性肿块17例(65.4％,17/26),伴有疼痛症状9例(36.4％,9/26).纳入分析的指标包括性别、年龄、肿瘤体积、Enneking外科分期、外科边界以及辅助化疗与否.肿瘤学评价包括局部复发率、转移率和生存分析.肿瘤体积与外科边界对局部复发率、辅助化疗对转移率的影响采用单因素分析;多因素分析局部复发率采用Logistic回归分析;生存因素采用Cox回归分析.结果 Enneking外科分期Ⅱ期14例,Ⅲ期12例.手术行边缘切除9例、广泛切除17例.随访时间5～226个月,平均45.9个月,中位随访时间为31个月,最终12例存活(46.2％).Ⅱ期和Ⅲ期病例五年生存率分别为79.5％和23.4％.局部复发7例(26.9％),广泛切除与边缘切除复发率的差异有统计学意义.Ⅱ期患者接受与未接受化疗转移发生率的差异无统计学意义.肿瘤直径＞5 cm和＜5cm的患者五年生存率分别为40.7％和80.0％.Logistic回归分析显示外科边界与局部复发率明显相关,Cox回归分析表明Enneking外科分期和肿瘤体积是死亡的独立预后因素.结论 腺泡状软组织肉瘤典型表现为无痛包块,易发生早期转移;外科边界是局部复发率的独立预后因素;Enneking外科分期与肿瘤体积是影响生存的独立危险因素;辅助化疗对于控制新发转移率和提高生存率的无明显改善.

abstractsObjective To explore clinical characteristics of alveolar soft part sarcoma(ASPS) and prognosis factors related to surgical treatment.Methods This study retrospectively analyzed 29 ASPS consecutive cases between Sep.1982 to Sep.2010,including 18 males and 11 females,with average 24.4 and median 21 years old (ranging 9 to 58).26 patients with surgery were enrolled in this study,with 23 cases primarily ocurred in soft tissue and 3 cases in bone.There were 17 cases with painless mass (65.4％,17/26) and 9 cases with pain (36.4％,9/26).Demographics,tumor size,stage,surgical margin,adjuvant chemotherapy provided,local recurrence,metastatic rate and overall survival probability were evaluated.We used multivariate analysis of logistic regression and Cox regression for local recurrence and survival rate respectively,and univariate analysis for tumor size,surgical margin,adjuvant chemotherapy and local recurrence.Results According to the Enneking classification,we distinguished stage Ⅱ 14 cases and stage Ⅲ 12 cases.Marginal excision was obtained in 9 cases as well as wide resection in 17 patients.The average and median follow-up time was 45.9 and 31 months (ranging 5 to 226) respectively,12 cases eventually survived (46.2％).The 5-year survival rates for stage Ⅱ and Ⅲ cases were 79.5％ and 23.4％ respectively.We found significant difference for local recurrence with surgical margin.However,there was no significant difference for prevention of metastatic progression with or without adjuvant chemotherapy.For tumor size,5-year survival rate of ＞ 5 cm and ＜ 5 cm group were 40.7％ and 80.0％ respectively.Multivariate analysis of logistic regression showed the surgical marginal was the only significant risk factor for local recurrence,while Cox regression showed both stage and tumor size were independent prognostic indicators for survival.Conclusion Although presenting as a slowly growing and painless mass,ASPS is an aggressive tumor with high risk of metastasis.Prognosis of ASPS is basically related to the characteristics of tumor size,surgical stage and quality of surgery.With limited improvement of adjuvant chemotherapy for metastasis control and survival,new agents are eagerly needed to complement surgery to eradicate this disease.