About Carcinoid Tumors

Overview of Neuroendocrine Tumors

Neuroendocrine tumors (NET) are classically divided into 2 categories: carcinoid tumors and pancreatic neuroendocrine tumors (PNET).1 Carcinoid tumors are derived from enterochromaffin cells throughout the gastrointestinal (GI) and bronchopulmonary systems.2,3 The most common sites for primary GI carcinoid tumors are the colon, rectum, and appendix3 (Figure 1). Approximately 9.4% of NET are PNET.4

Figure 1. Distribution of Carcinoid Tumors by Site3,4

Carcinoid Site Images

NET can also be located in the parathyroid, adrenal gland, pituitary glands, ovary, heart, and ear.1,5

NET were once considered to be rare neoplasms; however, over the past 30 years, there has been a 5-fold increase in their incidence.2,6 The annual age-adjusted incidence of NET is 5.25 per 100,0006; it is 5.35 per 100,000 in men compared with 4.76 per 100,000 in women.7 NET are more common in individuals over 50 years of age7, as well as in white patients (81%).6 The prevalence is 35 cases per 100,000 in the United States population (103,312), making NET the second most common Gl malignancy after colorectal cancer.6,8

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  1. Oberg K, Castellano D. Current knowledge on diagnosis and staging of neuroendocrine tumors. Cancer Metastasis Rev. 2011;30(suppl 1):3-7.
  2. Modlin IM, Oberg K, Chung DC et al. Gastroenteropancreatic neuroendocrine tumours. Lancet Oncol. 2008;9:61-72.
  3. Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer. 2003;97:934-959.
  4. Caldarella A, Crocetti E, Paci E. Distribution, incidence, and prognosis in neuroendocrine tumors: a population based study from a cancer registry:[published online April 9, 2011]. Pathol Oncol Res. 2011. doi: 10.1007/s12253-011-9382-y.
  5. National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology:neuroendocrine tumors. V1. https://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Accessed June 27, 2016.
  6. Yao JC, Hassan M, Phan A et al. One hundred years after "carcinoid": epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol. 2008;26:3063-3072.
  7. Oberg K, Akerstrom G, Rindi G, Jelic S. Neuroendocrine gastroenteropancreatic tumours: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2010;21 Suppl 5:v223-v227.
  8. National Cancer Institute. Surveillance, epidemiology, and end results (SEER) stat fact sheets. http://seer.cancer.gov/csr/1975_2013/results_merged/topic_prevalence.pdf.  Accessed June 27, 2016.