Epidemiology of Neuroendocrine TumoursTaal B.G.a · Visser O.b
aDepartment of Gastroenterology, Netherlands Cancer Institute/Antoni van Leeuwenhoek Hospital and bComprehensive Cancer Centre Amsterdam, Amsterdam, The Netherlands
Netherlands Cancer Institute/Antoni van Leeuwenhoek Hospital
NL–1066 CX Amsterdam (The Netherlands)
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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.
© 2004 S. Karger AG, Basel
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