摘要目的 探讨发生于外耳道的腺样囊性癌(adenoid cystic carcinoma,ACC)的临床病理学特征,及其预后相关情况.方法 收集2006年1月至2017年12月间解放军总医院和解放军总医院海南分院诊断的63例外耳道ACC患者的临床及随访资料,分析临床病理学特征及影响预后的相关因素.结果 (1)本组患者男性28例,女性35例,首次诊断时平均年龄48.9岁(22～81岁).筛状型35例(晚期15例),管状型14例(晚期8例),实性型14例(晚期9例),实性型相比于筛状型和管状型晚期患者数量相对较多,但差异无统计学意义(P>0.05).(2)随访到的57例患者,平均随访时间为62.4个月(时间范围2～228个月).复发的33例中,局部复发18例,远处转移15例,平均复发时间40.6个月(2～204个月).死亡的9例患者中,早期患者2例(首次治疗后48及102个月死亡),晚期患者7例(首次治疗后9～228个月死亡).(3)随访的57例患者中37例存在神经侵犯,其中筛状型、管状型及实性型分别为21、4及12例,早期及晚期患者分别为15和22例,差异均具有统计学意义(P<0.05);37例神经侵犯的患者中31例存在耳痛,差异无统计学意义(P>0.05).(4)57例患者均行手术治疗,肿物切除或联合颞骨部分切除45例,复发30例,肿物联合腮腺浅叶切除12例,复发3例,差异具有统计学意义(P<0.05).19例行术后辅助放疗,其中早期患者7例,复发2例;晚期患者12例,复发8例,差异无统计学意义(P>0.05).结论 发生于外耳道的ACC容易复发,首次手术时进行腮腺浅叶的切除对于预防肿瘤复发是必要的.术后辅助放疗对于早期患者具有一定疗效,但并不能明显降低肿瘤复发率.神经侵犯易发生于晚期患者,筛状型、实性型相对于管状型也更容易发生神经侵犯,然而神经侵犯与耳痛并无显著相关性.

abstractsObjective To investigate clinicopathologic features and prognosis of adenoid cystic carcinoma ( ACC) involving external auditory meatus. Methods The clinical presentation and follow-up data of 63 patients with ACC of external auditory canal were collected from January 2006 to February 2017 at PLA General Hospital and Hainan Branch of PLA General Hospital. The clinicopathologic features and prognostic factors of external auditory canal ACC were analyzed. Results ( 1) There were 28 males and 35 females and the average age of the first diagnosis was 48. 9 years ( 22-81 years ) . The tumors showed cribriform pattern in 35 cases ( 15 cases of late stage) , tubular pattern in 14 cases ( 8 cases of late stage) , and solid pattern in 14 cases (9 cases of late stage). Cases with solid pattern was relatively more frequent than that of cribriform pattern and tubular pattern, but the difference was not statistically significant ( P>0. 05) . ( 2 ) The average follow-up time was 62. 4 months ( 2-228 months ) in the 57 available cases. Among the 33 cases with recurrence, 18 cases had local recurrence and 15 cases had distant metastasis. The mean recurrence time was 40. 6 months ( 2-204 months) . Nine patients died of ACC:2 cases in early stage ( died at 48 and 102 months after the first treatment) , 7 cases in late stage and 57 with ( died at 9,30,32, 60,72,94 and 228 months) . ( 3) Among the 37 patients with perineural invasion, there were 21 cases of&nbsp;cribriform pattern, 4 cases of tubular pattern and 12 cases of solid pattern;the number of cases in early stage and late stage were 15 and 22, respectively;and the differences were statistically significant ( P<0. 05) . In addition, 31 cases had otalgia among the 37 patients with perineural invasion, where differences were not significant ( P>0. 05) . ( 4) Thirty of 45 cases with tumor resection or partial resection of temporal bone had recurrence, whereas 3 of 12 cases of tumor combined with superficial lobectomy of parotid gland had recurrence. The difference was statistically significant ( P<0. 05) . Postoperative adjuvant radiotherapy was given in 19 cases, including 7 cases of early stage ( 2 cases of recurrence ) , and 12 cases of late stage (8 cases of recurrence),among which there was no significant difference (P>0. 05). Conclusions ACC occurring in external auditory canal frequently recurs. Superficial parotid lobectomy at the first operation is necessary to prevent tumor recurrence. Postoperative adjuvant radiotherapy has certain curative effect on patients with early stage tumor, but it does not affect the recurrence rate. Patients at late stage are more prone to perineural invasion than those in early stage. In addition, cribriform and solid patterns are more common that tubular pattern, and there is no significant correlation between perineural invasion and otalgia.