Pancreatic neuroendocrine tumors (PNET) may develop sporadically or in the context of hereditary syndromes. In patients with multiple endocrine neoplasia type 1 (MEN1), PNET is the leading cause of death. Our aim was to compare the mortality risk in sporadic and MEN1-related PNETs and identify high-risk populations. A retrospective Surveillance, Epidemiology, and End Results database analysis of patients with PNET was used. Patients with MEN1 were defined by syn/metachronous pituitary adenoma. Clinical data were retrieved, and all-cause mortality (ACM) risk was compared in univariate and multivariable analyses. The cohort included 569 patients (46.6% males) with sporadic (n=542) and MEN1-related (n=27) PNETs. Age at diagnosis of MEN1-related PNET was significantly younger than with sporadic PNETs (mean age 49.2±16.7 vs. 61.6±12.7 years, respectively; p < 0.001). Survival analysis showed a trend for a better outcome in patients with MEN1-related vs. sporadic PNET (Log-rank, p=0.09) and in subgroup analysis for patients with advanced disease (p=0.08). Furthermore, among patients followed expectantly, those with MEN1-related PNET had lower ACM risk than their sporadic counterparts (p=0.08). Multivariable analysis demonstrated lower ACM risk in patients diagnosed with MEN1 (hazard ratio 0.35, 95% confidence interval 0.11–1.2, p=0.09), further supporting the trend detected in the univariate analysis. In conclusion, our study demonstrates the distinct clinical profile of patients with MEN1-related PNET compared to sporadic disease and emphasizes the expertise required to accurately manage patients with PNET in this rare context. The cautious decision-making required before embarking on surgical intervention is further emphasized in this robust analysis of a large cancer database.

中文翻译：

---

散发性和多发性内分泌肿瘤 1 型相关胰腺神经内分泌肿瘤的不同预后因素

胰腺神经内分泌肿瘤 (PNET) 可能散发性或在遗传性综合征的背景下发展。在多发性内分泌肿瘤 1 型 (MEN1) 患者中，PNET 是导致死亡的主要原因。我们的目的是比较散发性和 MEN1 相关 PNET 的死亡风险，并确定高危人群。使用了 PNET 患者的回顾性监测、流行病学和最终结果数据库分析。MEN1 患者被定义为同步/异时垂体腺瘤。检索临床数据，并在单变量和多变量分析中比较全因死亡率 (ACM) 风险。该队列包括 569 名散发 (n=542) 和 MEN1 相关 (n=27) PNET 的患者（46.6% 男性）。MEN1 相关 PNET 的诊断年龄明显小于散发性 PNET（平均年龄 49.2±16.7 岁与 61.6±12.7 岁，分别; p < 0.001)。生存分析显示，MEN1 相关患者与散发性 PNET 患者（Log-rank，p=0.09）和晚期疾病患者的亚组分析（p=0.08）有更好结果的趋势。此外，在预期随访的患者中，患有 MEN1 相关 PNET 的患者的 ACM 风险低于散发的患者（p = 0.08）。多变量分析表明诊断为 MEN1 的患者 ACM 风险较低（风险比 0.35，95% 置信区间 0.11-1.2，p=0.09），进一步支持单变量分析中检测到的趋势。总之，我们的研究表明，与散发性疾病相比，MEN1 相关 PNET 患者具有独特的临床特征，并强调了在这种罕见情况下准确管理 PNET 患者所需的专业知识。