Importance:Neuroendocrine tumors (NETs) have traditionally been considered rare (orphan) diseases; however, improvements in diagnostic methods and heightened awareness about NETs may have altered their epidemiologic profile in the U.S.Objective:To evaluate trends in incidence, prevalence, and survival of NETs in the United States from 2000 to 2021 using updated data from the SEER-22 registry.Method:This population-based, retrospective cohort study used the SEER-22 database for incidence, prevalence, and survival analyses. Data included 231,659 patients diagnosed with NETs between 2000 and 2021. Age-adjusted incidence, limited-duration prevalence, 5-year overall survival (OS), and hazard ratios (HRs) for survival by grade, stage, year of diagnosis, race, and primary site were investigated.Results:From 2000 to 2021, NET incidence nearly doubled, from 4.6 to 8.2 per 100,000 persons. The prevalence in 2021 reached 0.064%, with grade 1 tumors showing the steepest increase. Five-year OS was 77.4% overall and highest among localized NETs (82.3%) and grade 1 tumors (80.0%). Multivariable analysis showed improved survival over time (HR for 2015–2021 vs. 2000–2004, 0.92; 95% CI, 0.89–0.95). Black and American Indian/Alaska Native patients had significantly worse outcomes than White patients.Conclusions and Relevance:NETs are no longer orphan diseases based on current U.S. incidence and prevalence trends. With increasing survival and patient numbers, there is an urgent need for expanded multidisciplinary NET care and research efforts focused on survivorship and quality of life.
重要性：神经内分泌肿瘤（NETs）传统上被视为罕见（孤儿）疾病；然而，诊断方法的改进及对NETs认知度的提升可能已改变了其在美国的流行病学特征。 目的：利用SEER-22登记系统的最新数据，评估2000年至2021年美国NETs发病率、患病率及生存率的趋势。 方法：这项基于人群的回顾性队列研究采用SEER-22数据库进行发病率、患病率和生存率分析。数据涵盖2000年至2021年间确诊的231,659例NETs患者。研究分析了年龄调整发病率、有限期患病率、5年总生存率（OS），并按肿瘤分级、分期、诊断年份、种族及原发部位计算了生存风险比（HRs）。 结果：2000年至2021年间，NETs发病率从每10万人4.6例增至8.2例，几乎翻倍。2021年患病率达0.064%，其中1级肿瘤增幅最为显著。总体5年OS为77.4%，局部NETs（82.3%）和1级肿瘤（80.0%）生存率最高。多变量分析显示生存率随时间推移有所改善（2015–2021年 vs. 2000–2004年HR=0.92；95% CI 0.89–0.95）。黑人和美国印第安/阿拉斯加原住民患者的预后显著差于白人患者。 结论与相关性：基于当前美国的发病率和患病率趋势，NETs已不再属于孤儿疾病。随着生存率和患者数量的持续增长，亟需扩大NETs多学科诊疗体系，并加强针对患者生存质量与长期照护的研究投入。
肿瘤(癌症)患者之家