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Hépato-Gastro & Oncologie Digestive

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Epidemiologic characteristics of endocrine tumors of the digestive tract Volume 16, special issue 3, octobre 2009

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Authors
Inserm UMR 866, registre bourguignon des cancers digestifs, faculté de médecine, 7, boulevard Jeanne-d’Arc, 21000 Dijon, France

The epidemiologic characteristics of malignant endocrine tumours of the digestive tract (MDET) are poorly defined, both because they are rare and because their clinical and anatomic features are very diverse. They represented around 1 % of all incident digestive cancers. Age standardised incidence rates are esteemed around 1/100,000 inhabitants. They increased over time in both sexes. The categorization of malignant digestive endocrine tumours (MDETs) in the most recent International Histological Classification of Tumours1 pools digestive carcinoids with pancreatic endocrine tumours. Most endocrine tumours are well differentiated. Small cell MDETs, considered as particularly aggressive, are extremely rare. Mean age at diagnosis of MDET was slightly lower than age at diagnosis for other digestive cancers. Overall, in Europe approximately half of the patients with MDET survive five years after the initial diagnosis. Prognosis varies with tumour differentiation, anatomic site and histological type. There are significant differences in survival from MDET among European countries, independently of other prognostic factors. Well-differentiated MDETs are slow growing tumours (relative survival of 50 % at 5 years), whereas small cell MDETs are highly aggressive (relative survival of 5 % at 5 years). Stage at diagnosis is advanced when considering a general population.