Objective To analyze the clinicopathological features,treatment,and prognosis of adenoid cystic carcinoma (ACC) of trachea and bronchus. Methods Collected information of 11 cases of tracheal and bronchial ACC and observed the condition of the patients,analyzed the data as well as related literature. Results 11 cases included 3 male and 8 female pa-tients,the median age was 45 years. Cough,bloody sputum and progressive dyspnea were main symptoms. The tumors were lo-cated in central airway. Bronchoscopy showed that the neoplasms sprout out of airway or mucosal swelling caused airway steno-sis. Chest CT scanning showed irregular thickening wall or mass shadow with soft-tissue density. Microscopically , the tumor cells presented cribriform,tubular or solid growth pattern. 8 cases underwent surgery,including 5 cases with residual cancer in the trachea and bronchus. 3 of 5 patients with residual cancer were further treated by post-operative radiotherapy. Post-operative 5-year disease-free survival rate was 100%(4/4). The patients with residual cancer, within 4 years after surgical excision and further radiotherapy,did not appear recurrence and metastasis. 2 cases treated with radiochemotherapy and chemotherapy alone had survived for 14 months and 48 months without metastasis. Conclusion ACC of trachea and bronchus is a low grade ma-lignant tumor,which is slow in progress and is easy to be misdiagnosed. The vast majority of these tumors occur in the central airway,and the symptom is characterized by progressive dyspnea. Bronchoscopy and biopsy, CT scanning and image reconstruc-tion can determine the nature,scope of the lesions,and the treatment plan. ACC should be treated with surgery in combination with radiotherapy or radiotherapy alone. If the patient has airway obstruction and lose the chance of operation ,interventional therapy can be chosen.%目的：分析气管、支气管腺样囊性癌的临床病理特征、治疗及预后，提高对该病的认识与诊治水平。方法收集11例气管、支气管腺样囊性癌的临床病理资料、诊治情况，随访患者的病情变化及生存信息，结合文献进行分析。结果11例患者中，男3例，女8例，中位年龄45岁，主要症状为咳嗽、血痰及进行性加重的呼吸困难。肿瘤均位于中央气道，内镜下表现为隆起型肿物或气道粘膜肿胀肥厚，CT见管壁不规则增厚或软组织密度肿块影。组织学见肿瘤细胞排列成腺管状、筛状或实性团状。行手术治疗8例，其中5例切缘有癌残留者行放疗3例。术后5年无病生存率为100%（4/4），癌残留者术后放疗后4年均无复发转移。行姑息性放化疗或单纯化疗各1例，分别已生存14个月及48个月，均无转移。结论气管、支气管腺样囊性癌是一种好发于中央气道的低度恶性肿瘤，进展慢，易误诊，其进行性呼吸困难表现较特异，内镜检查并活检、CT扫描及其图像后处理对确定病变性质、范围及治疗方案有价值。治疗宜采用手术和放疗或单纯放疗，支气管镜下介入治疗对气道梗阻严重而不能手术的患者也是一个选择。
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