Han J, Gao X Z, Wei J G, Xie Y L, Liu Y Q, Li W C, Li S L
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.
Department of Pathology, Shaoxing People's Hospital, Zhejiang Province, Shaoxing 312000, China.
Zhonghua Bing Li Xue Za Zhi. 2019 Mar 8;48(3):204-208. doi: 10.3760/cma.j.issn.0529-5807.2019.03.007.
To investigate the clinicopathological features and prognostic indicators of primary pulmonary adenoid cystic carcinoma. 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. 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 (0.05). 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.
探讨原发性肺腺样囊性癌的临床病理特征及预后指标。收集2011年8月至2017年12月郑州大学第一附属医院收治的59例原发性肺腺样囊性癌病例。所有病例均采用苏木精-伊红染色和免疫组织化学方法进行回顾性研究。回顾临床病理特征，并采用Kaplan-Meier法和Cox回归模型进行患者生存分析。对59例研究病例中的15例进行了表皮生长因子受体（EGFR）、KRAS、BRAF基因状态分析。59例患者中，男性25例，女性34例，男女比例为1.0:1.4。患者年龄范围为29至81岁，平均年龄55岁。肿瘤最大直径范围为1.0至9.6 cm，平均直径2.8 cm。15例（25.4%）患者为吸烟者，44例（74.6%）患者为非吸烟者。肿瘤主要发生于气管（28/59，47.5%）、左主支气管（7/59，11.9%）和右支气管（5/59，8.5%）。大体上，肿瘤边界清楚，为灰白色结节。显微镜下肿瘤细胞小且均匀，呈管状、筛状和实性排列。免疫组织化学显示肿瘤细胞CK7、S-100蛋白、Sox-10、CD117和p63呈阳性。TTF1仅2例呈阳性，Ki-67指数范围为3%至40%。18例（30.5%）为Ⅰ级，26例（40.1%）为Ⅱ级，15例（25.4%）为Ⅲ级。总体而言，Ⅰ、Ⅱ、Ⅲ、Ⅳ期患者分别为39例（66.1%）、7例（11.9%）、10例（16.9%）和3例（5.1%）。23例（39.0%）患者接受了手术治疗，3例（5.1%）患者接受了手术联合放疗，9例（15.2%）患者接受了手术联合化疗，24例（40.7%）患者仅接受了化疗。15例检测病例中未检测到EGFR、KRAS和BRAF突变。第1、3、5年的总生存率分别为94.9%、86.4%和84.7%。预后分析显示患者年龄和肿瘤大小与生存具有统计学相关性（P<0.05）。大多数原发性肺腺样囊性癌患者处于临床早期，预后良好。肿瘤大小和患者年龄是独立的预后指标。
