原发胃肠间质瘤1027例诊治分析

Diagnosis and treatment of primary gastrointestinal stromal tumors:An analysis of 1027 cases

目的分析和探索华中科技大学同济医学院附属协和医院胃肠外科13年间原发胃肠间质瘤(GIST)的诊治情况。方法回顾分析2005年1月至2017年12月华中科技大学同济医学院附属协和医院诊治的1027例原发GIST病人临床病理及随访资料。结果本研究共纳入1027例GIST病人,男性563例(54.8%),女性464例(45.2%),中位年龄为57(19~90)岁。13年间新诊治的病例数分别为13、27、28、64、66、79、67、81、85、100、134、145、138例,逐年增长。肿瘤原发部位位于食管9例(0.9%),胃548例(53.4%),十二指肠102例(9.9%),空回肠216例(21.0%),结直肠51例(5.0%),胃肠道外(肠系膜、腹膜后、腹盆腔等)101例(9.8%)。根据改良美国国立卫生研究院(NIH)分级,其中极低危223例(21.7%),低危292例(28.4%),中危113例(11.0%),高危399例(38.9%)。512例中高危病人中,284例(55.5%)接受了基因检测。1027例病人均接受手术切除,其中630例(61.3%)为传统开放手术,295例(28.7%)为腹腔镜手术,102例(10.0%)为内镜切除。849例(82.7%)病人获得有效随访,中位随访时间42(3~150)个月;中高危病人中284例(55.5%)术后接受伊马替尼辅助治疗,中位辅助治疗时间为36(6~120)个月。1、3、5年总体无复发存活率分别为99.9%、92.7%、83.4%;1、3、5年总存活率分别为100.0%、97.6%、90.1%。根据危险度分级,极低危、低危、中危及高危病人其5年无复发存活率分别为100.0%、95.2%、85.2%、62.5%;5年总存活率分别为100.0%、96.5%、89.3%、78.4%。结论本研究是目前我国原发GIST病例数最多的单中心回顾性研究。13年间GIST诊治例数逐年增长,但基因突变检测率、术后辅助治疗率及总体疗效仍有待进一步提高。大宗样本资料可为进一步开展多中心回顾性及前瞻性研究提供可靠数据。

Objective To analyze and explore the diagnosis and treatment of primary gastrointestinal stromal tumor （GIST） in our center during the past 13 years. Methods The clinicopathologieal and follow-up data of 1027 patients with primary GIST diagnosed in Department of Gastrointestinal Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology from January 2005 to December 2017 were analyzed retrospectively. Results There were 563 male （54.8%） and 464 female （45.2%） cases with a median age of 57 （19-90） years. The number of newly diagnosed cases in 13 years was 13, 27, 28, 64, 66, 79, 67, 81, 85, 100, 134, 145 and 138, respectively, showing an increasing trend year by year. Primary GIST sites were esophagus in 9 （0.9%） cases, stomach in 548 （53.4%） cases, duodenum in 102 （9.9%） cases, jejunum and ileum in 216 （21.0%） cases, colon and rectum in 51 （5.0%） cases, and extra-gastrointestinal tract （including mesentery, retroperitoneum, abdominopelvic cavity, etc） in 101 （9.8%） cases. According to the modified National Institute of Health consensus criteria, 223 （21.7%） cases were very low risk, 292 （28.4%） cases were low risk, 113 （11.0%） cases were intermediate risk, 399 （38.9%） cases were high risk. Among the 512 patients with intermediate or high risk, 284 （55.5%） cases received gene detection. All the 1027 patients underwent surgical resection. According to the operation procedure, 630 （61.3% ） cases underwent laparotomy, 295 （28.7% ） cases underwent laparoscopic surgery, and 102 （10.0%） cases underwent endoscopic surgery. The majority of the patients （n=849, 82.7%） underwent follow-up for a median of 42 （3-150） months; and 284 （55.5%） patients of intermediate or high risk received adjuvant therapy with imatinib, with a median duration of 36 （6-120） months. The 1-, 3-, 5-year recurrence-free survival （RFS） were 99.9% , 92.7% , 83.4%, respectively; and the 1-, 3-, 5-year overall survival （OS） were 100.0%, 97.6%, 90.1%. The 5-year RFS rates in very low, low, intermediate, and high risk patients were 100% , 95.2% , 85.2% , 62.5%, respectively; and the 5-year OS rates were 100% , 96.5% , 89.3% , 78.4%. Conclusion The retrospective study presents the largest single institutional data of GIST in China. In the past 13 years, the number of GIST cases in the authors＇ center has increased year by year, but the detection rate of gene mutation, the rate of postoperative adjuvant therapy and the overall efficacy still need to be further improved. Furthermore, the bulk sample data can provide reliable data for further multieenter retrospective and prospective researches.