早发性胰腺神经内分泌肿瘤（EOPanNENs）的发病率、临床病理特征、治疗模式和生存情况尚未得到很好的探究。在2000年至2018年间，从SEER数据库中识别了被诊断为胰腺神经内分泌肿瘤（PanNENs）的患者。EOPanNENs定义为患者年龄小于50岁的诊断，而其它被定义为晚发性胰腺神经内分泌肿瘤（LOPanNENs）。我们的研究分析了发病率、临床特征、管理和预后。对EOPanNENs和LOPanNENs分别进行多因素分析以确定影响总体生存（OS）的因素。共纳入了5172名PanNENs患者：EOPanNENs队列中有1267名（24.5％），LOPanNENs队列中有3905名（75.5％）。随着年龄的增长，晚发性病例的年龄调整发病率显著增加，而早发性病例则相对稳定。与LOPanNEN相比，EOPanNENs更容易为女性，未婚，并具有更好的肿瘤分化。值得注意的是，早发性患者出现淋巴结受累的比率更高，且更可能接受手术治疗。对于局部-区域性疾病的治疗，手术单独治疗是过去二十年中最常用的方案。对于远程阶段，手术和化疗的组合更加常用。与EOPanNENs相比，PanNENs生存的风险因素与LOPanNENs更相关。EOPanNENs的OS和癌症特异性生存（CSS）明显更好。进一步分析表明，EOPanNENs ≤2cm与EOPanNENs>2cm相比，具有更有利的生存结局。EOPanNENs是与LOPanNENs具有临床上罕见和独特的实体。随着治疗的加强和肿瘤分化的改善，EOPanNENs队列的优势主要来自于缓慢的疾病进程。有必要对这种疾病亚组的特征进行进一步的研究。版权所有©2023 Yang、Liu、Leng、Liu和Shi。

The incidence, clinicopathologic characteristics, treatment patterns, and survival of early-onset pancreatic neuroendocrine neoplasms (EOPanNENs) have not been well explored.Patients diagnosed with PanNENs were identified from the SEER database between 2000 and 2018. EOPanNENs were defined as diagnosis in patients aged less than 50 years, while the remaining were defined as later-onset pancreatic neuroendocrine neoplasms (LOPanNENs). Incidence, clinical features, management, and prognosis were analyzed in our study. Multivariable analyses were performed to identify factors associated with overall survival (OS) in EOPanNENs and LOPanNENs, respectively.A total of 5172 patients with PanNENs were included: 1267 (24.5%) in the EOPanNENs cohort and 3905 (75.5%) in the LOPanNENs cohort. The age-adjusted incidence rate significantly increased among later-onset cases, while it remained relatively stable in early-onset cases. EOPanNENs were more frequently to be female, unmarried, and with better tumor differentiation compared with LOPanNENs. Of note, early-onset patients presented with a higher rate of lymph node involvement, and they were more likely to receive surgical treatment. For local-regional disease at presentation, surgery alone was the most frequently used regimen over the last two decades. With regard to distant stage, a combination of surgery and chemotherapy was more often utilized. Risk factors for PanNENs survival were more correlated with LOPanNENs compared with EOPanNENs. The OS and cancer-specific survival (CSS) were significantly better in the EOPanNENs group. Further analyses showed that EOPanNENs ≤ 2cm were associated with more favorable survival outcomes than EOPanNENs>2cm.EOPanNENs are a clinically rare and distinct entity from LOPanNENs. The advantages in survival for the EOPanNENs cohort over time were largely driven by the indolent clinical courses including better tumor differentiation and intensified surgical treatment. Further investigations are warranted to better understand the characteristics of this disease subgroup.Copyright © 2023 Yang, Liu, Leng, Liu and Shi.