外科手术切除胃中危胃肠间质瘤临床病理特征和预后因素的回顾性研究  

Clinicopathological features and prognostic factors of gastric intermediate-risk gastrointestinal stromal tumor after surgical resection:a retrospective study

作　　者：袁伟 黄雯 任磊 梁怀予 都向阳 傅敏 徐晨 方勇 沈坤堂 侯英勇 Yuan Wei;Huang Wen;Ren Lei;Liang Huaiyu;Du Xiangyang;Fu Min;Xu Chen;Fang Yong;Shen Kuntang;Hou Yingyong(Department of Pathology,Zhongshan Hospital,Fudan University,Shanghai 200032,China;Department of General Surgery,Zhongshan Hospital,Fudan University,Shanghai 200032,China)

机构地区：[1]复旦大学附属中山医院病理科,上海200032 [2]复旦大学附属中山医院普外科,上海200032

出　　处：《中华病理学杂志》2023年第4期384-389,共6页Chinese Journal of Pathology

基　　金：上海市科学技术委员会科研计划项目(19MC1911000);复旦大学附属中山医院临床研究专项基金(2020ZSLC01)。

摘　　要：目的探讨真实世界中外科手术切除胃中危胃肠间质瘤(gastrointestinal stromal tumor,GIST)的临床病理特征、治疗过程和预后,为今后临床实践的决策和研究提供参考。方法收集复旦大学附属中山医院1996年1月至2019年12月间诊断为胃中危GIST病例,记录临床病理特征、治疗经过和结局。结果360例胃中危GIST,男性190例,女性170例;平均年龄59岁;平均最大径5.9 cm;247例行基因检测(247/360,68.6%),其中KIT突变198例(80.2%)、PDGFRA突变26例(10.5%)、KIT/PDGFRA野生型23例(9.3%);按照12项指标的中山方法,非恶性239例,恶性121例(低度恶性103例,中度恶性15例,高度恶性3例)。获访241例,55例(22.8%)接受伊马替尼辅助治疗。10例进展(进展率4.1%),1例死亡(为PDGFRA突变,病死率0.4%),5年无瘤生存率96.0%,5年总生存率99.6%。此组胃中危GIST中,有无伊马替尼辅助治疗,在总体人群、KIT突变组、PDGFRA突变组、野生型组、非恶性组以及恶性组,无瘤生存率差异均无统计学意义(P>0.05)。但恶性组与非恶性组相比,在总体人群(P<0.01)、伊马替尼治疗组(P=0.044)、无伊马替尼治疗组(P<0.01)无瘤生存率差异均有统计学意义。KIT突变恶性患者,伊马替尼辅助治疗,无瘤生存率潜在获益(P=0.241)。结论胃中危GIST涵盖从良性到高度恶性的异质性生物学行为谱系,不能视为同一危险度的疾病,可进一步良恶性分层,以非恶性和低度恶性为主。外科手术切除总体疾病进展率低,真实世界数据显示术后现有伊马替尼治疗方案无明显获益。KIT基因突变恶性患者辅助治疗潜在延长无瘤生存率,GIST良恶性和基因突变综合分析对临床决策更有帮助。Objective To investigate the clinicopathological features,treatment and prognosis of gastric intermediate-risk gastrointestinal stromal tumor(GIST),so as to provide a reference for clinical management and further research.Methods A retrospective observational study of patients with gastric intermediate-risk GIST,who underwent surgical resection between January 1996 and December 2019 at Zhongshan Hospital of Fudan University,was carried out.Results Totally,360 patients with a median age of 59 years were included.There were 190 males and 170 females with median tumor diameter of 5.9 cm.Routine genetic testing was performed in 247 cases(68.6%,247/360),and 198 cases(80.2%)showed KIT mutation,26 cases(10.5%)showed PDGFRA mutation,and 23 cases were wild-type GIST.According to"Zhongshan Method"(including 12 parameters),there were 121 malignant and 239 non-malignant cases.Complete follow-up data were available in 241 patients;55 patients(22.8%)received imatinib therapy,10 patients(4.1%)experienced tumor progression,and one patient(PDGFRA mutation,0.4%)died.Disease-free survival(DFS)and overall survival rate at 5 years was 96.0%and 99.6%,respectively.Among the intermediate-risk GIST,there was no difference in DFS between the overall population,KIT mutation,PDGFRA mutation,wild-type,non-malignant and malignant subgroups(all P>0.05).However,the non-malignancy/malignancy analysis showed that there were significant differences in DFS among the overall population(P<0.01),imatinib treatment group(P=0.044)and no imatinib treatment group(P<0.01).Adjuvant imatinib resulted in potential survival benefit for KIT mutated malignant and intermediate-risk GIST in DFS(P=0.241).Conclusions Gastric intermediate-risk GIST shows a heterogeneous biologic behavior spectrum from benign to highly malignant.It can be further classified into benign and malignant,mainly nonmalignant and low-grade malignant.The overall disease progression rate after surgical resection is low,and real-world data show that there is no significant benefit from imatinib

关 键 词：伊马替尼 胃肠间质瘤 无瘤生存率 外科手术切除 低度恶性 临床决策 预后因素 良恶性 

分 类 号：R735[医药卫生—肿瘤]