[Associated gastroenteropancreatic neuroendocrine tumours to familiar syndromes].

Aproximately 5-10% of neuroendocrine tumours (NETs) of the gastroenteropancreatic system (GEP) have an hereditary background. The known hereditary syndromes include: multiple endocrine neoplasia type 1 (MEN 1), von Hippel Lindau disease (VHL), neurofibromatosis type 1 (NF 1) and tuberous sclerosis complex (TSC). This review discusses for each of these syndromes the: genes involved and specifics types of mutations, disease prevalence, affected neuroendocrine tissues and related clinical syndromes, and special morphological features of NETs on each of these syndromes.