摘要目的 探讨原发肺腺样囊性癌的临床病理特征,并对其预后及影响因素进行分析.方法 收集郑州大学第一附属医院2011年8月至2017年12月诊断的原发肺腺样囊性癌病例59例,进行常规HE染色和免疫组织化学染色,总结临床病理特征,并进行随访,采用Kaplan?Meier法及Cox风险回归模型进行生存相关的数据分析.并对15例进行表皮生长因子受体(EGFR)、KRAS及BRAF基因检测.结果 原发肺腺样囊性癌59例中,男25例,女34例,男女比例为1.0∶1.4,年龄29～81岁,平均年龄55岁,肿瘤最大径1.0～9.6 cm,平均2.8 cm.吸烟患者15例(25.4%),不吸烟患者44例(74.6%).肿瘤主要发生在气管28例(47.5%),左主支气管7例(11.9%),右主支气管5例(8.5%).大体表现为气管或者支气管内灰白色结节状肿物,显微镜下肿瘤细胞小而一致,排列呈管状、筛状或者实体状.免疫组织化学染色显示肿瘤表达CK7、S?100蛋白、Sox10、CD117、p63,2例病例甲状腺转录因子1(TTF1)阳性,Ki?67阳性指数3%～40%.肿瘤分级Ⅰ级18例(30.5%),Ⅱ级26例(40.1%),Ⅲ级15例(25.4%).TNM分期Ⅰ期39例(66.1%),Ⅱ期7例(11.9%),Ⅲ期10例(16.9%),Ⅳ期3例(5.1%).治疗方法包括单纯手术治疗23例(39.0%),手术辅助放疗3例(5.1%),手术辅助化疗9例(15.2%),单纯化疗24例(40.7%).EGFR、KRAS及BRAF基因均未检测到突变.第1、3、5年的总体生存率分别为94.9%、86.4%及84.7%.预后分析显示患者的年龄和肿瘤的大小对患者预后影响有统计学意义(P<0.05).结论 原发肺腺样囊性癌患者多数为早期病变,预后较好,肿瘤的大小和患者的年龄是影响生存的独立预后因素.

abstractsObjective To investigate the clinicopathological features and prognostic indicators of primary pulmonary adenoid cystic carcinoma. Methods Fifty?nine cases of primary pulmonary adenoid cystic carcinoma were collected from August 2011 to December 2017 at the First Affiliated Hospital of Zhengzhou University. All cases were retrospectively studied by hematoxylin?eosin staining and immunohistochemistry. The clinicopathological features were reviewed and patient survival analysis was performed using Kaplan?Meier method and Cox regression model. Status of epidermal growth factor receptor (EGFR), KRAS, BRAF genes was analyzed in 15 of the 59 study cases. Results Among 59 cases, there were 25 males and 34 females with male to female ratio of 1.0 to 1.4. The patient age ranged from 29 to 81 years with a mean age of 55 years. The tumor max diameters ranged from 1.0 to 9.6 cm with an average diameter of 2.8 cm. Fifteen (25.4%) patients were smokers while 44 patients (74.6%) were non?smokers. Tumors predominantly occurred in the trachea (28/59,47.5%), the left main bronchus (7/59,11.9%) and the right bronchus (5/59,8.5%). Grossly, the tumors were well circumscribed, greyish?white nodules. Microscopically the tumor cells were small and uniform, and arranged in tubular, cribriform, and solid patterns. Immunohistochemistry showed that the tumor cells were positive for CK7, S?100 protein, Sox?10, CD117 and p63. TTF1 was only positive in 2 cases and Ki?67 index ranged from 3% to 40%. Eighteen cases (30.5%) were gradeⅠ, 26 cases (40.1%) grade Ⅱ, and 15 cases (25.4%) grade Ⅲ. Overall, 39 cases (66.1%), 7 cases (11.9%), 10 cases (16.9%), and 3 cases (5.1%) were at stages Ⅰ, Ⅱ, Ⅲ, and Ⅳ, respectively. Twenty?three patients (39.0%) received surgical therapy, 3 patients (5.1%) surgery combined with radiotherapy, 9 patients (15.2%) surgery combined with chemotherapy, and 24 cases (40.7%) chemotherapy only. No mutation of EGFR, KRAS and BRAF was detected in all 15 tested cases. The overall survival rate at the first, third and fifth years was 94.9%, 86.4% and 84.7%, respectively. Prognostic analysis showed that patient′s age and tumor size were statistically associated with the survival (P<0.05). Conclusions Majority of the patients with primary pulmonary adenoid cystic carcinoma are at an early clinical stage with a favorable prognosis. The size of the tumor and the age of the patients are independent prognostic indicators.