Department of Hepatopancreatobiliary Surgery, Qingdao Municipal Hospital, Qingdao University, Qingdao, China.
Qingdao Hospital, University of Health and Rehabilitation Sciences (Qingdao Municipal Hospital), Qingdao, China.
Front Endocrinol (Lausanne). 2023 Apr 4;14:1025485. doi: 10.3389/fendo.2023.1025485. eCollection 2023.
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.
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充分探索。
本研究从 2000 年至 2018 年的 SEER 数据库中确定了诊断为胰腺神经内分泌肿瘤（PanNENs）的患者。EOPanNENs 定义为诊断时年龄<50 岁的患者，其余患者定义为晚发性胰腺神经内分泌肿瘤（LOPanNENs）。本研究分析了发病率、临床特征、治疗和预后。分别对 EOPanNENs 和 LOPanNENs 进行多变量分析，以确定与总生存期（OS）相关的因素。
共纳入 5172 例 PanNENs 患者：EOPanNENs 队列 1267 例（24.5%），LOPanNENs 队列 3905 例（75.5%）。年龄调整后的发病率在晚发性病例中显著增加，而在早发性病例中相对稳定。EOPanNENs 女性、未婚和肿瘤分化程度较好的比例高于 LOPanNENs。值得注意的是，早发性患者淋巴结受累率较高，更可能接受手术治疗。对于局部区域疾病，20 年来，单独手术是最常用的治疗方案。对于远处阶段，手术联合化疗更为常用。与 LOPanNENs 相比，PanNENs 生存的危险因素与 EOPanNENs 更为相关。EOPanNENs 组的 OS 和癌症特异性生存（CSS）明显更好。进一步分析显示，EOPanNENs≤2cm 比 EOPanNENs>2cm 有更好的生存获益。
EOPanNENs 是一种临床罕见且与 LOPanNENs 不同的实体。随着时间的推移，EOPanNENs 组的生存优势在很大程度上是由更好的肿瘤分化和强化手术治疗等较为惰性的临床过程驱动的。需要进一步研究以更好地了解这一疾病亚组的特征。
