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      Epidemiology of Neuroendocrine Tumours

      a , b


      S. Karger AG

      Neuroendocrine tumours, Epidemiology

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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.

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          Most cited references 8

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          Familial carcinoid tumors and subsequent cancers: a nation-wide epidemiologic study from Sweden.

          Carcinoids are rare neuroendocrine tumors, mainly located in the bowel, stomach and lung. Familial risks in carcinoid tumours are not well known apart from multiple endocrine neoplasia 1 (MEN1). We used the nation-wide Swedish Family-Cancer Database on 10.1 million individuals for assessment. Carcinoid tumors were retrieved from the Cancer Registry covering the years 1958-1998. The offspring generation, aged 0-66 years, accumulated 190 million person-years at risk. The age-adjusted incidence rates were 0.76 for men and 1.29/100,000 for women. Standardized incidence ratios (SIRs) were calculated for offspring when their parents had a carcinoid or any other cancer. When parents presented with carcinoids, SIRs for offspring were 4.35 (n = 8, 95% CI 1.86-7.89) for small intestinal and 4.65 (n = 4, 95% CI 1.21-10.32) for colon carcinoids. If both offspring and parents presented with small intestinal carcinoids, the SIR was 12.31 (n = 4, 95% CI 3.20-27.34). Offspring carcinoids were also increased if parents presented with bladder and endocrine gland tumors, the latter association probably partially due to MEN1. Risks for second cancers were increased, particularly at sites where familial risks were found, including carcinoids in the small intestine. Copyright 2001 Wiley-Liss, Inc.
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            Incidence trends and risk factors of carcinoid tumors

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              Epidemiology of carcinoid neoplasms in Vaud, Switzerland, 1974–97

              In Vaud, Switzerland, the incidence of carcinoids based on 218 malignant and 215 benign cases rose from 19.6/106in 1974–85 to 28.2/106in 1986–97, more so among males and malignant neoplasms. Lung was the commonest site for malignant and large intestine for benign carcinoids. Sixty-eight (16%) carcinoids had another neoplasm. © 2000 Cancer Research Campaign

                Author and article information

                S. Karger AG
                October 2004
                15 October 2004
                : 80
                : Suppl 1
                : 3-7
                aDepartment of Gastroenterology, Netherlands Cancer Institute/Antoni van Leeuwenhoek Hospital and bComprehensive Cancer Centre Amsterdam, Amsterdam, The Netherlands
                80731 Neuroendocrinology 2004;80(suppl 1):3–7
                © 2004 S. Karger AG, Basel

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                Page count
                Figures: 2, Tables: 2, References: 17, Pages: 5


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