Épidémiologie des tumeurs neuroendocrines digestives: la situation en France

Little is known about the epidemiology of digestive neuroendocrine tumours (NETs) in France. NETs remain a rare cancer, representing 1% of digestive cancers. In France, incidence rates are around 1.1/100,000 inhabitants in males and 0.9/ 100,000 in females. Incidence rates increased over time, with probably more than 1,000 newcases per year in France.Due to their relatively good prognosis, NETs are the second more prevalent digestive cancer after colorectal cancer. Most gastroentero-pancreatic NETs are well-differentiated (WD-NETs); poorly differentiated neuroendocrine carcinomas (PDNEC) account for less than 20% of the cases in most of the series. Among WD-NETs, the most frequent anatomical localisations are small bowel and pancreas. Functional NETs are rare (<20%);most of them are carcinoid tumours, insulinomas or gastrinomas. More than half NETs are metastatic at diagnosis, mainly in the liver. Tumour differentiation, histologic grade, anatomic site, and stage are themain prognostic factors.WD-NETs are slow growing tumours (relative survival of 55% at 5 years), whereas PDNEC are highly aggressive (relative survival of 4.5% at 5 years).