The incidence of carcinoid syndrome in a population in central Sweden was estimated as being approximately 0.5 per 100,000 population per year.[3]Norheim I, Oberg K, Theodorsson-Norheim E, et al. Malignant carcinoid tumors. An analysis of 103 patients with regard to tumor localization, hormone production, and survival. Ann Surg. 1987 Aug;206(2):115-25.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1493111
http://www.ncbi.nlm.nih.gov/pubmed/2440390?tool=bestpractice.com
Carcinoid syndrome occurs in patients with functional carcinoid tumours (i.e., symptoms are caused by hormonal release). There has been a clear rise in incidence of all gastroenteropancreatic neuroendocrine tumours (NETs), and specifically midgut carcinoid tumours, over the last two decades but the reasons for this are not clear.[4]Perez EA, Koniaris LG, Snell SE, et al. 7201 carcinoids: increasing incidence overall and disproportionate mortality in the elderly. World J Surg. 2007 May;31(5):1022-30.
http://www.ncbi.nlm.nih.gov/pubmed/17429568?tool=bestpractice.com
[5]Dasari A, Shen C, Halperin D, et al. Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol. 2017 Oct 1;3(10):1335-42.
https://jamanetwork.com/journals/jamaoncology/fullarticle/2621997
http://www.ncbi.nlm.nih.gov/pubmed/28448665?tool=bestpractice.com
Epidemiological data estimate the age-adjusted incidence of NETs in the US population at 6.98 per 100,000 of which 3.56 per 100,000 were gastroenteropancreatic NETs.[5]Dasari A, Shen C, Halperin D, et al. Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol. 2017 Oct 1;3(10):1335-42.
https://jamanetwork.com/journals/jamaoncology/fullarticle/2621997
http://www.ncbi.nlm.nih.gov/pubmed/28448665?tool=bestpractice.com
[6]Das S, Dasari A. Epidemiology, incidence, and prevalence of neuroendocrine neoplasms: are there global differences? Curr Oncol Rep. 2021 Mar 14;23(4):43.
https://www.doi.org/10.1007/s11912-021-01029-7
http://www.ncbi.nlm.nih.gov/pubmed/33719003?tool=bestpractice.com
In the UK the age-adjusted incidence of neuroendocrine neoplasia is estimated at 8.61 per 100,000.[7]White BE, Rous B, Chandrakumaran K, et al. Incidence and survival of neuroendocrine neoplasia in England 1995-2018: a retrospective, population-based study. Lancet Reg Health Eur. 2022 Dec;23:100510.
https://www.thelancet.com/journals/lanepe/article/PIIS2666-7762(22)00206-X/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/36176500?tool=bestpractice.com
The Surveillance, Epidemiology, and End Results (SEER) programme data suggest a slight female sex bias (52.7% women), a preponderance in the age group 65 years and over, and a greater incidence in white people than black people and Hispanics.[5]Dasari A, Shen C, Halperin D, et al. Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol. 2017 Oct 1;3(10):1335-42.
https://jamanetwork.com/journals/jamaoncology/fullarticle/2621997
http://www.ncbi.nlm.nih.gov/pubmed/28448665?tool=bestpractice.com
[8]Halperin DM, Shen C, Dasari A, et al. Frequency of carcinoid syndrome at neuroendocrine tumour diagnosis: a population-based study. Lancet Oncol. 2017 Apr;18(4):525-34.
https://pmc.ncbi.nlm.nih.gov/articles/PMC6066284
http://www.ncbi.nlm.nih.gov/pubmed/28238592?tool=bestpractice.com
Carcinoid tumours are the most common functional NET. Approximately 19% of patients with NETs have carcinoid syndrome. This proportion has increased over the last two decades.[8]Halperin DM, Shen C, Dasari A, et al. Frequency of carcinoid syndrome at neuroendocrine tumour diagnosis: a population-based study. Lancet Oncol. 2017 Apr;18(4):525-34.
https://pmc.ncbi.nlm.nih.gov/articles/PMC6066284
http://www.ncbi.nlm.nih.gov/pubmed/28238592?tool=bestpractice.com
While the incidence of NETs is relatively low at 8-9 per 100,000 population per year, the overall prevalence of this condition is much higher due to the relatively indolent nature of the disease.[9]Genus TSE, Bouvier C, Wong KF, et al. Impact of neuroendocrine morphology on cancer outcomes and stage at diagnosis: a UK nationwide cohort study 2013-2015. Br J Cancer. 2019 Nov;121(11):966-72.
https://www.doi.org/10.1038/s41416-019-0606-3
http://www.ncbi.nlm.nih.gov/pubmed/31649320?tool=bestpractice.com