ArticleJAMA network open2025
Epidemiology of Neuroendocrine Neoplasms in the US.
Article in JAMA network open, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 64 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
64 citing papers in PubMed, 1 synthesis or guideline pooled it.
- The incidence of gastric neoplastic lesions in autoimmune gastritis: a systematic review and meta-analysis.Surgical endoscopy · 2026Pooled it
- Cabozantinib and temozolomide in patients with advanced progressive neuroendocrine tumors: a phase 2 study.Nature communications · 2026Trial
- Axitinib and Long-Acting Octreotide in Advanced Extrapancreatic Neuroendocrine Tumors: A Randomized, Double-Blind, Placebo-Controlled, Phase III Clinical Trial (AXINET, GETNE 1107).Journal of clinical oncology : official journal of the American Society of Clinical Oncology · 2026Trial
- Multicenter Phase I Study of Triapine in Combination with [177Lu]Lutetium-Dotatate in Patients with Well-Differentiated Neuroendocrine Tumors (ETCTN 10388).Clinical cancer research : an official journal of the American Association for Cancer Research · 2026Article
- Alternative lengthening of telomeres in small well-differentiated pancreatic neuroendocrine tumors: independent validation and cross-platform concordance.Virchows Archiv : an international journal of pathology · 2026Article
- The Genomic Patterns of Well-Differentiated Pancreatic Neuroendocrine Tumors (NETs) Identify Sub-Sets for Rational Therapeutic Targeting.International journal of molecular sciences · 2026Article
- Evaluation of [¹⁸F]AlF-NOTA-octreotide PET/CT in routine clinical use: a retrospective analysis in 288 neuroendocrine tumor patients.European journal of nuclear medicine and molecular imaging · 2026Article
- [Rectal neuroendocrine tumors of the rectum: update on endoscopic and surgical treatment].Chirurgie (Heidelberg, Germany) · 2026Review
- Article
- Rare solid tumours as indicators of hereditary cancer syndromes.European journal of human genetics : EJHG · 2026Review
- Role of Interventional Oncology in the Management of Neuroendocrine Tumors: 2026 Update.Current oncology reports · 2026Review
- Article
- Article
- Humanized antibody-drug conjugates for Somatostatin receptor 2 (SSTR2)-positive tumor treatment.Molecular cancer therapeutics · 2026Article
- Review
- Single-Cell and Spatial Transcriptomics Reframe the Immunosuppressive Microenvironment of Neuroendocrine Neoplasms.Cancers · 2026Review
- Hepatic arterial embolization procedures in neuroendocrine tumors with carcinoid heart disease: a retrospective single-center study on safety and feasibility.Endocrine-related cancer · 2026Article
- Real-world presentation and outcomes of gastroenteropancreatic neuroendocrine neoplasms in Italy: findings from the nationwide Itanet prospective database.The Lancet regional health. Europe · 2026Article
- Tumor count and risk of lymph node metastasis in multiple rectal neuroendocrine tumors: an individual-level pooled analysis.World journal of surgical oncology · 2026Article
- Incidence and Survival of Gastro-Entero-Pancreatic Neuroendocrine Neoplasms in the Contemporary Era.JAMA network open · 2026Article
4 more citing papers are in PubMed but not listed here.
Corrections and comments
- Erratum issued
Authors and funding
8 authors.
Funding
Abstract
Importance: Neuroendocrine neoplasms (NENs) are increasing in incidence; prevalence and at the same time, practice patterns have also evolved, impacting classification and survival of these malignant neoplasms. However, updated epidemiological data are lacking. Objective: To define the epidemiological and survival trends of patients with NENs in the US. Design, Setting, and Participants: This cross-sectional study used data from the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) program on NEN cases from 1975 to 2021. Analysis of project data was conducted between August 2023 and August 2024. Main Outcomes and Measures: Annual age-adjusted incidence between 1975 and 2021, limited duration prevalence, and overall survival (OS) rates. Recent trends in survival were evaluated from 2000 to 2021 for the entire cohort as well as specific subgroups including distant stage gastrointestinal neuroendocrine tumors (NETs) and pancreatic NETs. Results: In this analysis of 145 447 NEN cases (mean [SD] age, 61.4 [14.7] years; 76 057 female [52.4%]), the age-adjusted incidence rate increased 5.2-fold from 1975 (1.64 per 100 000 persons; 95% CI, 1.43-1.87 per 100 000 persons) to 2021 (8.52 per 100 000 persons; 95% CI, 8.33-8.70 per 100 000 persons) except for a dip in 2020 likely related to the COVID pandemic. This increase occurred across all sites, stages, and grades but was most marked for localized stage neoplasms (13-fold; 1975: 0.40 per 100 000 persons [95% CI, 0.30-0.52]; 2021: 5.04 per 100 000 persons [95% CI, 4.78-5.30]), well-differentiated neoplasms (53-fold; 1975: 0.04 per 100 000 persons [95% CI, 0.02-0.09]; 2021: 2.30 per 100 000 persons [95% CI, 2.13-2.48]), and neoplasms with the appendix (12-fold; 1975: 0.11 per 100 000 persons [95% CI, 0.09-0.22]; 2021: 1.68 per 100 000 persons [95% CI, 1.39-1.78]) or rectum (12-fold; 1975: 0.11 per 100 000 persons [95% CI, 0.06-0.18]; 2021: 1.32 per 100 000 persons [95% CI, 1.19-1.46]) as primary sites. Since 2000 (SEER 17 registry), the sites with the highest incidence rates included lung (1.49 per 100 000 persons) and gastroenteropancreatic (GEP) NENs (6.1 per 100 000 persons); within GEP NENs, small bowel (1.4 per 100 000 persons) and pancreas (1.3 per 100 000 persons) were the most common sites. The estimated 20-year limited duration prevalence of NENs in the US on January 1, 2021, was 243 896 cases. OS for all NENs improved from the 2000-2006 period to the 2014-2021 period (hazard ratio [HR], 1.42; 95% CI, 1.38-1.45). In addition, other factors associated with survival included age, stage, grade, and primary site of origin. The median OS for all NENs was 11.8 years, and for distant-stage, well-differentiated neuroendocrine tumors it was 6.7 years with 10-year OS ranging from 17 410 patients (15.4%) with rectum as primary site to 17 505 patients (51.7%) with small bowel as primary site. Conclusions and Relevance: Incidence and prevalence of NENs continue to rise driven by increased diagnosis of early stage disease. Survival for NENs has also improved over time.
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