180例野生型胃肠间质瘤患者的临床病理特征分析

Analysis of clinicopathological characteristics in 180 patients with wild type gastrointestinal stromal tumors

目的：大样本分析中国野生型胃肠间质瘤（GIST）患者的临床病理特征。方法收集2001年10月至2013年9月于北京大学肿瘤医院就诊的180例野生型胃肠间质瘤患者的临床病理资料，分析野生型GIST患者的临床病理特征，并与同时期的临床病理资料较完整的513例突变型GIST患者进行比较。结果180例野生型GIST患者中，男女比例1∶1，中位年龄52．5（16～78）岁。肿瘤原发于胃者82例（45．6％），小肠43例（23．9％），结直肠21例（11．7％），另有34例（18．9％）患者肿瘤发生于腹腔、盆腔、网膜和肾周等其他部位。50岁以下患者中肿瘤长径大于5 cm的比例明显高于50岁以上患者[77．2％（61／79）比54．9％（50／91），P＜0．01]；且肿瘤大小与核分裂象具有显著关联（P＜0．01）。CD117阳性率为81．7％（138／169），DOG-1阳性率为75．6％（62／82），CD34阳性率为74．7％（115／154）；3种因子均于胃GIST中阳性率最高。与突变型GIST患者比较，野生型GIST发生于年轻患者和原发于胃者比较更高，肿瘤相对更小，CD117和DOG-1阳性率相对较低（均P＜0．01）。结论野生型与突变型GIST在发病年龄、发病部位、肿瘤大小及CD117、DOG-1表达率方面存在明显差异，提示野生型GIST可能作为一个独立的亚型，在临床治疗中需特别关注。

Objective To investigate the clinicopathological characteristics of 180 patients with wild type gastrointestinal stromal tumors （GIST）. Methods The clinicopathological data of 180 patients with wild type GIST treated in the Peking University Cancer Hospital between October 2001 and September 2013 were analyzed and compared to 513 mutant patients. Results 180 patients were included in this study, and the median age 52.5 years （16-78 years）. The percentage of female was significantly higher than that of male among patients under the age of 40 . Primary tumor sites mainly included stomach （45.6%） and small intestine （23.9%）. The positive expression of CD117, DOG-1, and CD34 were 81.6%, 75.6%, and 74.7%, respectively, which were highest in stamach, secondly in small intestine. The percentage of tumor with length 〉 5 cm was higher in patients under the age of 50 compared to patients more than 50 years, and a significant correlation was found between tumor size and mitosis. Compared to mutant patients, wild type patients had the following features： younger, primary tumor site of stomach, tumor length ≤2 cm, and the lower positive expression of CD117 and DOG-1. Conclusions The significant differences in primary tumor sites, tumor size, and the positive expression of CD117/DOG-1 were found between wild type GIST and mutant GIST , which suggested