Epidemiology of Large Bowel Carcinoid Tumors in the USA: A Population-Based National Study
Background and Aims
Prior studies have shown that about 90% of all carcinoid tumors occur in the GI tract. However, epidemiological studies of these tumors have been limited by small sample size. Our aim was to obtain a more robust epidemiologic survey of large bowel carcinoids (LBC), using population-based data in order to more accurately identify risk factors for these tumors.
We used a commercial database (Explorys Inc, Cleveland, OH) which includes electronic health record data from 26 major integrated US healthcare systems. We identified all patients aged 18 and older who were diagnosed with LBC, excluding appendiceal carcinoids, between 1999 and 2018 based on Systematized Nomenclature Of Medicine—Clinical Terms (SNOMED-CT) and evaluated the prevalence of LBC. We also performed univariate analysis to describe age-, race-, and gender-based distributions and to identify potential risk factors.
Of the 62,817,650 individuals in the database, 4530 were identified to have LBC with an overall prevalence of 7.21/100,000. Individuals with LBC were more likely to be elderly (age > 65) [OR 2.17, CI 2.05–2.31, p < 0.0001], smokers [OR 3.25; 95% CI 3.00–3.53, p < 0.0001], have a history of alcohol use [OR 3.75; 95% CI 3.52–3.99, p < 0.0001], diabetes mellitus (DM) [OR 4.42; 95% CI 4.14–4.72, p < 0.0001], obesity [OR 1.58; 95% CI 1.43–1.74, p < 0.0001], family history of cancer [OR 8.06; 95% CI 7.47–8.71, p < 0.0001], and personal history of ulcerative colitis [OR 6.93; 95% CI 5.55–8.64, p < 0.0001] or Crohn’s disease [OR 6.45; 95% CI 5.24–7.95, p < 0.0001]. The prevalence of LBC was less among Caucasians compared to African–Americans [OR 0.57; 95% CI 0.53–0.61, p < 0.0001]. There was no statistically significant gender-based difference; men versus women [OR 1.02; 95% CI 0.96–1.08, p = 0.47]. The most common presenting symptoms included flushing, diarrhea, nausea, weight loss, and abdominal pain, while the most common GI associations included perforation, obstruction, hemorrhage, intussusception, and volvulus.
This is the largest epidemiological study evaluating the prevalence of LBC. We estimated the prevalence rate of LBC to be 7.21/100,000. The presence of significant risk factors with the clinical picture suspicious for a LBC should warrant thorough evaluation as these tumors can lead to life-threatening complications. Further studies are needed to better understand the mechanism of association between these risk factors and LBC.
KeywordsNeuroendocrine tumors Carcinoid tumors Large bowel carcinoids Epidemiology Prevalence
Large bowel carcinoids
Gastrointestinal neuroendocrine tumors
Surveillance, epidemiology, and end results
Health data gateway
Health information technology for economic and clinical health act
End results group
Third national cancer survey
National cancer institute
Electronic health record
International classification of diseases
Systematized nomenclature of medical terminology—clinical terms
PL, MAS, EM, GK, MMG, GI, GSC were involved in study conception and design. PL, MAS, EM, GK, MMG, GI, GSC contributed to acquisition of data. PL, MAS, EM, GK, MMG, GI, GSC helped in analysis and interpretation of data. PL, MAS, EM drafted the manuscript. MAS, EM, GI, GSC contributed to critical revision. PL, MAS, EM, GK, MMG, GI, GSC were involved in statistical analysis. Cooper obtained funding. GI, GSC helped in study supervision.
Supported in part by the Cleveland Digestive Disease Research Core Center (P30DK097948) and the Case Comprehensive Cancer Center (P30CA43703) (both to Cooper).
Compliance with Ethical Standards
Conflict of interest
There are no potential conflicts (financial, professional, or personal) to disclose by the other authors (Lal, Saleh, Mansoor, Isenberg, Khoudari, Gad).
- 4.Obendorfer S. Karzinoide tumoren des dunndarms. Frankf Z Pathol. 1907;1:425–432.Google Scholar
- 14.Arnold R. Introduction: definition, historical aspects, classification, staging, prognosis and therapeutic options. Best Pract Res Clin Endocrinol Metab. 2005;19:491–505.Google Scholar
- 16.Altman DG. Practical Statistics for Medical Research. Boca Raton: CRC Press; 1990.Google Scholar
- 25.Chen CC, Neugut AI, Rotterdam H. Risk factors for adenocarcinomas and malignant carcinoids of the small intestine: preliminary findings. Canc Epidemiol Biomark Prev. 1994;3:205–207.Google Scholar
- 28.Szabo GG, Barta Z, Kerekes L, Szakáll S. Association of carcinoid tumor of the appendix and Crohn disease (case report and review of the literature). Orv Hetil. 1999;140:1635–1639.Google Scholar
- 30.Barhoum M, Hutchins L, Fonseca VA. Intractable hypercalcemia due to a metastatic carcinoid secreting parathyroid hormone-related peptide and interleukin-6: response to octreotide. Am J Med Sci. 1999;318:203–205.Google Scholar
- 31.Kasprzak A, Przewoźna M, Surdyk-Zasada J, Zabel M. The expression of selected neuroendocrine markers and of anti-neoplastic cytokines (IL-2 and IL-12) in lung cancers. Folia Morphol (Warsz). 2003;62:497–499.Google Scholar
- 34.Abou Saleh M, Mansoor E, Anindo M, Isenberg G. Prevalence of small intestine carcinoid tumors: a US population-based study 2012–2017. Dig Dis Sci. 2018. (Epub ahead of print). doi: https://doi.org/10.1007/s10620-018-5402-z.