背景:原发性小肠腺癌(PSBA)的临床病理特征和预后,不包括十二指肠癌,由于它在日本的罕见性,仍然不确定。
方法:我们分析了354名患者的358名PSBA,2008年1月至2017年12月,在日本结肠和直肠癌协会下属的44个机构中。
结果:中位年龄为67岁(218名男性,61.6%)。平均肿瘤大小为49.9(7-100)mm。PSBA位点包括空肠(66.2%)和回肠(30.4%)。共有219例患者(61.9%)接受了诊断性小肠内镜检查,包括单气囊内窥镜检查,双气囊内窥镜检查,治疗前进行胶囊内镜检查。19例患者(5.4%)患有林奇综合征,272例患者(76.8%)在初始诊断时出现症状.阶段0,I,II,III,IV为5.4%,2.5%,27.1%,26.0%,和35.6%,分别。各阶段5年总生存率为92.3%,60.0%,75.9%,61.4%,和25.5%,分别,5年疾病特异性生存率(DSS)为100%,75.0%,84.1%,59.3%,和25.6%,分别。PSBA位于空肠的患者,在初始诊断或晚期临床阶段出现症状的患者预后较差.然而,使用Cox风险模型的多变量分析显示,临床分期是PSBA患者DSS的唯一重要预测因素。
结论:在PSBA患者中,76.8%的人在最初诊断时出现症状,经常在晚期被发现。在PSBA的早期阶段进行检测对于确保良好的预后很重要。
The clinicopathological features and prognosis of primary small bowel adenocarcinoma (PSBA), excluding duodenal cancer, remain undetermined due to its rarity in Japan.
We analyzed 354 patients with 358 PSBAs, between January 2008 and December 2017, at 44 institutions affiliated with the Japanese Society for Cancer of the Colon and Rectum.
The median age was 67 years (218 males, 61.6%). The average tumor size was 49.9 (7-100) mm. PSBA sites consisted of jejunum (66.2%) and ileum (30.4%). A total of 219 patients (61.9%) underwent diagnostic small bowel endoscopy, including single-balloon endoscopy, double-balloon endoscopy, and capsule endoscopy before treatment. Nineteen patients (5.4%) had Lynch syndrome, and 272 patients (76.8%) had symptoms at the initial diagnosis. The rates for stages 0, I, II, III, and IV were 5.4%, 2.5%, 27.1%, 26.0%, and 35.6%, respectively. The 5-year overall survival rates at each stage were 92.3%, 60.0%, 75.9%, 61.4%, and 25.5%, respectively, and the 5-year disease-specific survival (DSS) rates were 100%, 75.0%, 84.1%, 59.3%, and 25.6%, respectively. Patients with the PSBA located in the jejunum, with symptoms at the initial diagnosis or advanced clinical stage had a worse prognosis. However, multivariate analysis using Cox-hazard model revealed that clinical stage was the only significant predictor of DSS for patients with PSBA.
Of the patients with PSBA, 76.8% had symptoms at the initial diagnosis, which were often detected at an advanced stage. Detection during the early stages of PSBA is important to ensure a good prognosis.