对于早发性胃肠胰神经内分泌肿瘤（GEP-NENs），流行病学趋势和生存情况尚未受到充分的研究。本研究从“监测、流行病学和终点数据”的数据库中获得了1975年至2018年早发性GEP-NENs发病率和发病率相关死亡率的趋势，并根据年龄、性别、种族、肿瘤部位、分期和分级进行了分层。相关人口数据用于确定早发性GEP-NENs患者的总体生存率（OS）和独立预后因素。本研究纳入了17299例早发性GEP-NENs患者。结果显示，1975年至2018年早发性GEP-NENs的发病率（每年增加5.95%，95%置信区间（CI）为5.75-6.14%）和发病率相关死亡率（每年增加4.24%，95% CI为3.92-4.56%）呈上升趋势，增幅高于晚发性GEP-NENs（发病率：每年增加4.45%，95% CI为4.38-4.53；发病率相关死亡率：每年增加4.13%，95% CI为3.89-4.37）。除了分期不明患者外，所有年龄、种族、肿瘤部位、分级和分期的患者的发病率均上升。与晚发性GEP-NENs相比，早发性GEP-NENs患者中女性比例更高（54.5% vs. 49.0%，p < 0.001），肿瘤分化良好（31.1% vs. 28.0%，p < 0.05），局部病变更多（55.2% vs. 46.7%，p < 0.05）。此外，无论肿瘤部位、分级还是分期，与晚发性GEP-NENs相比，早发性GEP-NENs的总体生存率更好（p < 0.0001）。多变量生存分析确定了种族、婚姻状况、分期、分级、化疗和原发部位与早发性GEP-NENs患者的OS显著相关。 早发性GEP-NENs的发病率和发病率相关死亡率稳步增加，增幅高于晚发性GEP-NENs。早发性GEP-NENs和晚发性GEP-NENs群体的临床特征和生存率有所不同。种族、婚姻状况、分期、分级、化疗和原发部位是早发性GEP-NENs的独立预后因素。有必要进一步研究该疾病亚群的特征。 版权所有 © 2023 Yao, Hu, Jiang, Fan, Yuan, Shi and Lin.

The epidemiologic trends and survival related to early-onset gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) have not been well explored.Trends in the incidence and incidence-based mortality of early-onset GEP-NENs between 1975 and 2018 were obtained from the Surveillance, Epidemiology, and End Results database, and were stratified by age, sex, race, tumor site, stage, and grade. Associated population data were used to determine overall survival (OS) and independent prognostic factors for patients with early-onset GEP-NENs.A total of 17299 patients diagnosed with early-onset GEP-NENs were included in this study. Results revealed an increase in the incidence (5.95% per year, 95% confidence interval (CI), 5.75-6.14%) and incidence-based mortality (4.24% per year, 95% CI, 3.92-4.56%) for early-onset GEP-NENs from 1975 to 2018, with higher rates of increase than those of later-onset GEP-NENs (incidence: 4.45% per year, 95% CI, 4.38-4.53; incidence-based mortality: 4.13% per year, 95% CI, 3.89-4.37; respectively). Increases in incidence were observed across all age, races, tumor sites, grades, and stages, except for patients with unknown stage. Compared to those with later-onset GEP-NENs, a higher proportion of female gender (54.5% vs. 49.0%, p <0.001), well-differentiated tumor (31.1% vs. 28.0%, p <0.05), and localized disease (55.2% vs. 46.7%, p <0.05) were observed in the cohort of patients with early-onset GEP-NENs. Moreover, early-onset GEP-NENs exhibited a superior overall survival in comparison to later-onset GEP-NENs, irrespective of tumor site, grade, or stage (p <0.0001). Multivariable survival analysis identified that race, marital status, stage, grade, chemotherapy, and primary site were significantly correlated with OS in individuals with early-onset GEP-NENs.The incidence and incidence-based mortality rates of early-onset GEP-NENs have steadily increased over time, with higher rates of increase than those of later-onset GEP-NENs. The clinical characteristics and survival were different between early-onset and later-onset GEP-NENs groups. Race, marital status, stage, grade, chemotherapy, and primary site were independent prognostic factors for early-onset GEP-NENs. Further investigations are warranted to better understand the characteristics of this disease subgroup.Copyright © 2023 Yao, Hu, Jiang, Fan, Yuan, Shi and Lin.