Mangion Jessica, Vassallo Josanne, Gruppetta Mark
Department of Medicine, Faculty of Medicine and Surgery, University of Malta, Mater Dei Hospital, Msida, Malta.
Neuroendocrine Clinic, Department of Medicine, Mater Dei Hospital, Msida, Malta.
Endocr Oncol. 2025 Aug 5;5(1):e250039. doi: 10.1530/EO-25-0039. eCollection 2025 Jan.
Neuroendocrine neoplasms, though rare, have shown marked increases in global incidence and prevalence over the past decade, as demonstrated by cancer registry data and studies from specialised tertiary centres. However, it remains unclear whether this represents a true increase in incidence or enhanced detection capabilities across various countries. This review aims to analyse and discuss recently published data on the worldwide epidemiology of gastroenteropancreatic NEN, and bronchopulmonary NEN and explore potential trends and differences in incidence rates between large and small nations according to demographics, primary sites, grade and stage. Following PRISMA guidelines, 59 cohort studies published between 2014 and 2024 were analysed. Findings reveal mixed demographic patterns, with a slight male predominance in overall NEN incidence but site-specific variations influenced by genetic, hormonal, and environmental factors. Median age at diagnosis is 60 years, though appendiceal NENs typically affect younger individuals. Racial disparities were noted, with higher incidence rates among Black populations in the USA, though findings varied across studies. Globally, the incidence of NENs has increased, with the most notable surge reported in the USA between 1973 and 2012. Similar upward trends were observed in Europe, Asia, and Australia, though to different extents. However, variations in data collection methods present significant challenges for cross-national comparisons. The review highlights the need for standardised methodologies and further research to address gaps in understanding NEN epidemiology in smaller nations and underrepresented regions.
神经内分泌肿瘤虽然罕见，但癌症登记数据和专业三级中心的研究表明，在过去十年中其全球发病率和患病率显著上升。然而，尚不清楚这是发病率的真正上升还是各国检测能力提高所致。本综述旨在分析和讨论最近发表的关于胃肠胰神经内分泌肿瘤（Gastroenteropancreatic NEN）和支气管肺神经内分泌肿瘤（Bronchopulmonary NEN）全球流行病学的数据，并根据人口统计学、原发部位、分级和分期探讨大国和小国发病率的潜在趋势及差异。按照PRISMA指南，对2014年至2024年间发表的59项队列研究进行了分析。研究结果揭示了复杂的人口统计学模式，总体神经内分泌肿瘤发病率中男性略占优势，但存在受遗传、激素和环境因素影响的部位特异性差异。诊断时的中位年龄为60岁，不过阑尾神经内分泌肿瘤通常影响较年轻个体。研究发现了种族差异，美国黑人人群的发病率较高，但不同研究结果有所不同。全球范围内，神经内分泌肿瘤的发病率有所上升，1973年至2012年间美国的增长最为显著。欧洲、亚洲和澳大利亚也观察到类似的上升趋势，不过程度不同。然而，数据收集方法的差异给跨国比较带来了重大挑战。该综述强调需要标准化方法和进一步研究，以填补对小国和代表性不足地区神经内分泌肿瘤流行病学认识的空白。
