Epidemiology of neuroendocrine tumours.
Neuroendocrine tumours account for only 0.5% of all malignancies. The incidence is approximately 2/100,000 with a female preponderance under the age of 50 years due to appendiceal location. The main primary sites are the gastrointestinal tract (62-67%) and the lung (22-27%). Presentation with metastatic disease accounts for 12-22%. In the last decades, the incidence has been rising. This might be due to more awareness, improved diagnostic tools or a change in definition. Most neuroendocrine tumours are mainly sporadic, but association with the multiple endocrine neoplasia type 1 syndrome and clustering within families is known. Also an increased risk of secondary cancers has been reported, but numbers are small. The 5-year survival is mainly associated with stage: 93% in local disease, 74% in regional disease and 19% in metastatic disease. In metastatic disease, survival increased since 1992, when treatment with octreotide became largely available in The Netherlands.
Department of Gastroenterology, Netherlands Cancer Institute, Antoni van Leeuwenhoek Hospital, Amsterdam, The Netherlands. b.taal@nki.nl
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D000367:Age Factors; D016021:Epidemiologic Studies; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000401:mortality; D006801:Humans; D015994:Incidence; D008297:Male; D009367:Neoplasm Staging; D009426:Netherlands / Q000453:epidemiology; D018358:Neuroendocrine Tumors / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology* / Q000401:mortality; D011379:Prognosis; D012307:Risk Factors; D015996:Survival Rate; D013997:Time Factors