Incidence of gastroenteropancreatic neuroendocrine tumours: A systematic review of the literature

M. Fraenkel, M. Kim, A. Faggiano, W. W. De Herder, G. D. Valk

Research output: Contribution to journalReview articlepeer-review

224 Scopus citations


Based on the current medical literature, the worldwide incidence of neuroendocrine tumours (NETs) seems to have increased; however, a systematic literature overview is lacking. This study aimed to collect all available data on the incidence of gastroenteropancreatic (GEP)-NETs and characteristics of population to establish their epidemiology. A sensitive MEDLINE search was carried out. The papers were selected via a cascade process that restricted the initial pool of 7991 articles to 33, using predefined inclusion and exclusion criteria. Original articles evaluating the incidence of sporadic GEP-NETs in regional, institutional and national registries were considered. The majority of data originated from the US National Cancer Institute Surveillance, Epidemiology and End Results database and from national cancer registries in Western Europe. Generally, because of the retrospective nature of existing databases the outcomes of studies might be biased, which hinders the drawing of firm conclusions. The age-adjusted incidence of GEP-NETs has increased steadily over the past four decades (1973-2007), increasing 3.65-fold in the USA and 3.8- to 4.8-fold in the UK. Incidence has changed variably from one anatomical site to another. The greatest increase in incidence occurred for gastric and rectal NETs, while the smallest increase occurred for small intestine NETs. There were gender and racial differences, which differed site by site and, in some cases, changed over time. The incidence rates (IRs) of GEP-NETs have increased significantly in the last 40 years. Data are only available from North America,Western Europe and Japan. A siteby-site analysis revealed that the IRs of some NETs increased more than those of others.

Original languageEnglish
Pages (from-to)R153-R163
JournalEndocrine-Related Cancer
Issue number3
StatePublished - Jun 2014


  • Carcinoids
  • Epidemiology
  • Incidence
  • Neuroendocrine tumours


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