Background: Small intestinal neuroendocrine tumors (siNET) are one of the most common neuroendocrine neoplasms. Radical surgery is the only curative treatment. Method: We utilized a single-center study including consecutive patients diagnosed from 2000 to 2020 and followed them until death or the end of study. Disease-specific survival and recurrence-free survival (RFS) were investigated by Cox regression analyses with the inclusion of prognostic factors. Aims/primary outcomes: We identified three groups: (1) disease specific-survival in the total cohort (group1), (2) RFS and disease-specific survival after intended radical surgery (group2), (3) disease specific-survival in patients with unresectable disease or residual tumor after primary resection (group3). Results: In total, 615 patients, with a mean age (SD) 65 ± 11 years were included. Median (IQR) Ki-67 index was 4 (2–7)%. Median disease-specific survival in group1 was 130 months. Median RFS in group2 was 138 months with 5- and 10-year RFS rates of 72% and 59% with age, plasma chromogranin A (p-CgA) and Ki-67 index as prognostic factors. The ten year disease-specific survival rate in group2 was 86%. The median disease-specific survival in group3 was 85 months with age, Ki-67 index, p-CgA and primary tumor resection as prognostic factors. When proliferation was expressed by WHO grade, no difference was observed between G1 vs. G2 for any of the primary outcomes. Conclusions: Recurrence rates remained high 5–10 years after surgery (group2) supporting long-term follow-up. Median disease-specific survival in patient with unresectable disease (group3) was 7 years, with a favorable impact of primary tumor resection. Our data does not support the current grading system since no significant prognostic information was detected in G1 vs. G2 tumors.
背景:小肠神经内分泌肿瘤(siNET)是最常见的神经内分泌肿瘤之一。根治性手术是唯一可能治愈的治疗方法。方法:我们采用单中心研究,纳入2000年至2020年间连续诊断的患者,并随访至其死亡或研究结束。通过纳入预后因素的Cox回归分析,研究了疾病特异性生存期和无复发生存期(RFS)。目的/主要结局:我们确定了三组患者:(1)总队列的疾病特异性生存期(第1组),(2)计划性根治手术后患者的RFS和疾病特异性生存期(第2组),(3)患有不可切除疾病或初次切除后残留肿瘤患者的疾病特异性生存期(第3组)。结果:共纳入615例患者,平均年龄(标准差)为65±11岁。Ki-67指数中位数(四分位距)为4%(2-7%)。第1组的中位疾病特异性生存期为130个月。第2组的中位RFS为138个月,5年和10年RFS率分别为72%和59%,年龄、血浆嗜铬粒蛋白A(p-CgA)和Ki-67指数是预后因素。第2组的10年疾病特异性生存率为86%。第3组的中位疾病特异性生存期为85个月,年龄、Ki-67指数、p-CgA和原发肿瘤切除是预后因素。当用WHO分级表示增殖活性时,在任何主要结局指标上,G1级与G2级之间均未观察到差异。结论:术后5-10年复发率仍然很高(第2组),这支持了长期随访的必要性。不可切除疾病患者(第3组)的中位疾病特异性生存期为7年,原发肿瘤切除对其有积极影响。我们的数据不支持当前的分级系统,因为在G1级与G2级肿瘤之间未检测到显著的预后信息差异。