Department of Oncology, University of Alberta, Cross Cancer Institute, Edmonton, Alberta, Canada,
Neuroendocrinology. 2021;111(9):876-882. doi: 10.1159/000511812. Epub 2020 Sep 25.
The aim of the study was to provide a real-world, population-based assessment of the incidence and outcomes of neuroendocrine neoplasms (NENs) of unknown primary.
Surveillance, Epidemiology, and End Results registry was accessed, and cases with NENs of unknown primary were reviewed. Rates of NENs diagnosis 1975-2017 according to primary tumor site were also reviewed. Survival outcomes of patients with NENs of unknown primary compared to metastatic NENs with known primary were determined through Kaplan-Meier estimates and multivariable Cox regression analysis. Overall and cancer-specific survival analyses were stratified by primary site and histology (neuroendocrine tumor vs. neuroendocrine carcinoma).
A total of 3,550 cases (7%) were diagnosed with NENs of unknown primary within the study duration. The annual percent change for NENs of unknown primary was 3.4 (95% CI: 2.6-4.2). Within the cohort of metastatic neuroendocrine tumor patients (carcinoid tumor histology), the following factors were associated with a lower risk of death; younger age (HR: 0.477; 95% CI: 0.443-0.513), female sex (HR: 0.922; 95% CI: 0.860-0.989), and small intestinal primary (HR for unknown primary vs. small intestinal primary: 1.532; 95% CI: 1.408-1.668). Within the cohort of metastatic neuroendocrine carcinoma, the following factors were associated with a lower risk of death in this cohort; younger age (HR: 0.646; 95% CI: 0.612-0.681), female sex (HR: 0.843; 95% CI: 0.801-0.888), and small intestinal primary (HR for unknown primary vs. small intestinal primary: 2.961; 95% CI: 2.586-3.391).
The diagnosis of NENs of unknown primary has increased across the past 4 decades. Outcomes of individuals with metastatic small intestinal NENs seem to be better than those with metastatic NENs of unknown primary (for both carcinoid tumors and neuroendocrine carcinomas).
本研究旨在提供一项基于人群的真实世界评估，以评估无法确定原发灶的神经内分泌肿瘤（NENs）的发病率和结局。
我们查阅了监测、流行病学和最终结果（SEER）登记数据库，并对无法确定原发灶的 NENs 病例进行了回顾性分析。还根据原发灶部位分析了 1975 年至 2017 年 NENs 诊断率。通过 Kaplan-Meier 估计和多变量 Cox 回归分析，确定无法确定原发灶的 NENs 患者与已知原发灶的转移性 NENs 患者的生存结局。总生存和癌症特异性生存分析按原发灶部位和组织学（神经内分泌肿瘤 vs. 神经内分泌癌）进行分层。
在研究期间，共诊断出 3550 例（7%）无法确定原发灶的 NENs。无法确定原发灶的 NENs 的年变化率为 3.4%（95%CI：2.6-4.2）。在转移性神经内分泌瘤（类癌肿瘤组织学）患者队列中，以下因素与较低的死亡风险相关：年龄较小（HR：0.477；95%CI：0.443-0.513）、女性（HR：0.922；95%CI：0.860-0.989）和小肠原发灶（无法确定原发灶与小肠原发灶相比的 HR：1.532；95%CI：1.408-1.668）。在转移性神经内分泌癌队列中，以下因素与该队列较低的死亡风险相关：年龄较小（HR：0.646；95%CI：0.612-0.681）、女性（HR：0.843；95%CI：0.801-0.888）和小肠原发灶（无法确定原发灶与小肠原发灶相比的 HR：2.961；95%CI：2.586-3.391）。
在过去的 40 年中，无法确定原发灶的 NENs 的诊断有所增加。小肠转移性 NENs 患者的结局似乎优于无法确定原发灶的转移性 NENs 患者（类癌肿瘤和神经内分泌癌均如此）。
