Epidemiology of Neuroendocrine Neoplasms in the US
An erratum to this article was published (JAMA Netw Open, 3 Nov 2025 ↗)
Authors' conclusion
Incidence and prevalence of NENs continue to rise driven by increased diagnosis of early stage disease. Survival for NENs has also improved over time.
Study context
- Population
- Patients with neuroendocrine neoplasms in the US, Surveillance, Epidemiology, and End Results (SEER) program, 1975–2021; mean age 61.4 years, 52.4% female
- Participants
- 145,447 NEN cases
- Outcome
- Annual age-adjusted incidence, limited duration prevalence and OS rates
- Follow-up
- 1975–2021
Methodological detailspopulation-based registry (SEER), Analysis conducted between August 2023 and August 2024
- Population-based registry (SEER)
- Analysis conducted between August 2023 and August 2024
Main finding
As reported in the abstract
Age-adjusted incidence rate of NENs · 1975 to 2021
- Age-adjusted incidence, 1975
- 1.64 per 100 000 persons (95% CI 1.43-1.87)
- Age-adjusted incidence, 2021
- 8.52 per 100 000 persons (95% CI 8.33-8.70)
- Increase in incidence 1975 to 2021, except a dip in 2020 likely related to the COVID pandemic
- 5.2-fold
- Estimated 20-year limited duration prevalence on January 1, 2021
- 243 896 cases
- Median overall survival (OS), all neuroendocrine neoplasms (NENs)
- 11.8 years
Observational data: associations, not effects.
Further findings
| Outcome | HR (95% CI) | |
|---|---|---|
| OS, all NENs, 2000-2006vs 2014-2021 periodOS improved over time | 1.42 (1.38–1.45) | |
| 0.512Hazard ratio (log scale) |
Incidence increase by subgroup (1975 vs 2021), per 100 000 persons
- Localized stage: 0.40 [95% CI 0.30-0.52] to 5.04 [95% CI 4.78-5.30]
- 13-fold
- Well-differentiated: 0.04 [95% CI 0.02-0.09] to 2.30 [95% CI 2.13-2.48]
- 53-fold
- Appendix primary: 0.11 [95% CI 0.09-0.22] to 1.68 [95% CI 1.39-1.78]
- 12-fold
- Rectum primary: 0.11 [95% CI 0.06-0.18] to 1.32 [95% CI 1.19-1.46]
- 12-fold
- Increase occurred across all sites, stages and grades
Incidence by site since 2000 (SEER 17), per 100 000 persons
- Lung
- 1.49
- Gastroenteropancreatic (GEP) NENs
- 6.1
- Small bowel (within GEP)
- 1.4
- Pancreas (within GEP)
- 1.3
OS, all NENs, 2000-2006 vs 2014-2021 period
- Other factors associated with survival: age, stage, grade, primary site
OS, distant-stage well-differentiated neuroendocrine tumors (NETs)
- Median OS
- 6.7 years
- 10-year OS, rectum primary (17 410 patients)
- 15.4%
- 10-year OS, small bowel primary (17 505 patients)
- 51.7%
Original publication
Epidemiology of Neuroendocrine Neoplasms in the US. JAMA Netw Open. Published 2 June 2025. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →