Endocrine oncology

Incidence and Survival of Gastro-Entero-Pancreatic Neuroendocrine Neoplasms in the Contemporary Era

JAMA Netw OpenPublished Population-based retrospective cohort study

Authors' conclusion

This cohort study of GEP-NENs found that incidence has continued to rise, largely due to increased diagnosis of nonmetastatic disease; OS and CSS were good but heterogeneous, influenced by demographic and disease-specific factors. These contemporary, population-based estimates provide essential evidence to support patient counselling, survivorship planning, health-system resource allocation, and the design of future NEN research.

Study context

Population
Adults diagnosed with incident gastro-entero-pancreatic neuroendocrine neoplasms (GEP-NENs) in Ontario, Canada (2000–2023); median (IQR) age 61 (50–71) years, 6228 (48.4%) female
Participants
12,877 adults
Outcome
Annual incidence per 100 000 population; OS (Kaplan-Meier) and CSS (cumulative incidence, noncancer deaths as competing risk)
Methodological detailslinked administrative datasets
  • Linked administrative datasets

Main finding

As reported in the abstract

Incidence and survival of GEP-NENs

Overall incidence per 100 000, 2000 to 2023

1.83 to 7.46

Nonmetastatic disease incidence per 100 000 per year (6.5-fold rise); minimal change in metastatic presentations

1.48 to 7.18

Ten-year overall survival (OS), all GEP-NENs

63.3%

95% CI 62.3%–64.3%

Ten-year cancer-specific survival (CSS), all GEP-NENs

76.4%

95% CI 75.6%–77.3%

Observational data: associations, not effects.

Further findings

Synchronous metastases

Individuals with synchronous metastases (16.5%)
2313

Incidence by site

Incidence highest for small intestine, then rectum, pancreas, appendix, stomach, colon; largest increases in gastric and pancreatic NENs

10-year CSS by metastases at diagnosis

Without metastases at diagnosis
exceeded 90.0%
With metastases at diagnosis
51.3% (95% CI, 49.7%-52.8%)

Survival by site and associated factors

Most favorable survival for rectal and appendiceal NENs, least for colonic and pancreatic NENs
Advancing age, lower socioeconomic status and metastases at diagnosis independently associated with worse OS and CSS; female sex with superior OS and CSS

Original publication

Incidence and Survival of Gastro-Entero-Pancreatic Neuroendocrine Neoplasms in the Contemporary Era. JAMA Netw Open. Published 1 June 2026. doi:10.1001/jamanetworkopen.2026.16492

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