[Genetics of neuroendocrine tumors]

Genetics of neuroendocrine tumours is mainly related to major inherited syndromes which predispose to the development of tumours in endocrine glands. Clinical and genetic studies on multiple endocrine neoplasia (MEN) syndromes show the diversity and complexity of cellular and genetic pathways involved in endocrine tumor genesis. Nevertheless, we might expect that recent cloning of most of the genes involved in MEN and related syndromes, has been a powerful and historical step to help clinicians in differential diagnosis of MEN and related diseases, mainly in cases which show familial association of various types of endocrine tumors. To date, 4 to 6 genes may be commonly analyzed and a single patient diagnosed for a specific syndrome, thus leading to a better clinical and therapeutical follow-up for patients and related predisposed individuals.