目的提高外耳道腺样囊性癌的诊疗水平。方法回顾性分析10例外耳道腺样囊性癌的临床资料。复习相关文献并作综合分析。结果所有患者均得到以手术切除为主的综合治疗,随访时间2～17年。术后复发4例,死亡2例,临床治愈4例。结论外耳道腺样囊性癌不易早期确诊,有必要对此病提高警惕。以手术治疗为主的综合治疗成为目前主要治疗手段。由于肿瘤境界不清,如何达到彻底切除值得探讨。 Objective To improve the diagnosis and treatment of external auditory canal cystic carcinoma. Methods The clinical data of 10 cases of extracanal adenoid cystic carcinoma were retrospectively analyzed. Review the relevant literature and make a comprehensive analysis. Results All patients were treated with surgical resection-based comprehensive treatment, follow-up time of 2 to 17 years. Postoperative recurrence in 4 cases, 2 patients died of clinical cure in 4 cases. Conclusions Ear canal adenoid cystic carcinoma is not easy to diagnose early, it is necessary to be vigilant on the disease. Surgical treatment-based comprehensive treatment has become the main treatment. As the tumor is not clear, how to achieve complete resection is worth exploring.