气管腺样囊性癌的临床治疗经验分析

Clinical experience in treatment of primary tracheal adenoid cystic carcinoma

目的探讨气管腺样囊性癌（ACC）最佳的合理治疗模式。方法回顾性分析1995年1月至2014年12月上海市胸科医院诊治的原发性气管ACC患者的资料。采用Cox多因素回归分析ACC患者总生存期和无病生存期的影响闶素。结果共109例患者纳入分析，其中男性53例（48．6％），女性56例（51．4％）；ACC患者的年龄为21～71岁，平均年龄（46．9±9．0）岁，中位年龄46岁；切除的肿瘤平均直径和气管平均长度分别为（28．9±7．4）mm和（32．9±7．4）mm；总体镜下切端阳性（R1）比例为84．4％（92／109）。所有患者的5年和10年总生存率及无病生存率分别为88．7％和43．2％，及62．0％和20．0％。根据切缘m性与否以及术后是否进行辅助放疗进一步分组分析：在总生存率方面，切缘阴性未进行术后辅助放疗的患者（R0／0）显著优于切缘阳性并进行术后放疗（R1／1）组以及切缘阳性未进行术后放疗（RI／0）组（χ2=4．410，P=0．036；χ2－8．448，P=0．004）；而R1／1组与R1／0组的总生存率比较差异无统计学意义（χ2-=1.690，P=0．194）。在无病生存率方面，R0／0组和R1／1组均优于R1／0组（χ2＝7．808，P－0．005；χ2=9．907，P=0．002），而R0／0组与R1／1组之间差异无统计学意义（χ2=2．210，P=0．137）。结论气管ACC外科切除后生存时间较满意；气管ACC浸润范围广，R0切除率低；R1切除的患者辅助放疗能有效改善预后；手术切除或术后联合放疗可作为较局限的该类气管肿瘤的首选治疗方案。

Objective To investigate the optimal treatment strategy for primary tracheal adenoid cystic carcinoma （ACC）. Methods The clinical data of patients with primary tracheal ACC treated between January 1995 and December 2014 in Shanghai Chest Hospital were retrospectively analyzed. Cox multivariate analysis was adopted to investigate the influencing factors of overall survival and disease-free survival in patients with ACC. Results A total of 109 patients were identified, including 53 males and 56 females with an average age of （46.9±9.0） years （median 46 years, range 21-71 years）. The mean resected tumor size and tracheal length were （28.9 ± 7.4） mm and （32.9 ± 7.4） mm, respectively. Patients with microscopically positive margin accounted for 84.4% （92/109）. Five and 10 years overall survival （OS） and disease free survival （DFS） were 88. 7% and 43. 2%, and 62. 0% and 20. 0%, respectively. The overall survival in patients with negative incision margin and no postoperative radiotherapy（R0/0） was significantly higher than that in patients with positive incision margin and postoperative radiotherapy（R1/1） and that in patients with positive incision margin and no postoperative radiotherapy（R1/0） （χ2 =4. 410, P=0. 036; χ2 = 8. 448, P= 0.004）. However, there was no significant difference in overall survival between R1/1 group and R1/0 group （χ2 = 1. 690, P=0. 194）. The disease-free survival in R0/0 group and R1/1 group was significantly higher than that in group R1/ 0 （χ2 = 1. 690, P = 0. 005 ;） χ2=9. 907 P = 0. 002）, while there was no significant difference between R0/ 0 group and R1/1 group （χ2=2.210, P=0. 137）. Conclusions R1 resection and postoperative radiotherapy can yield favorable outcomes of tracheal ACC, which can serve as the optimal regimen for this disease.