[Recent data on molecular genetics of neuroendocrine tumors]

Genetic studies of neuroendocrine tumors were mainly performed on the basis of major syndromes predisposing to endocrine and associated proliferative lesions. Carcinoid tumors and tumors of the pancreatic islet cells occur in Multiple Endocrine Neoplasia type 1, Neurofibromatosis type I, Von Hippel-Lindau disease, Tuberous Sclerosis, suggesting that alterations of the major genes responsible for these genetic syndromes are crucial mechanisms in the neuroendocrine pathogenesis. Tumoral progression and metastasis result from secondary genetic events or deregulation involving specific genes, oncogenes, suppressor genes, and growth factors. Mechanisms involving mitosis, cell cycle, and cellular adhesion might be relevant in the metastatic process. This report is an overview of experimental data on this topic and attempt to understand the major events inducing a malignant evolution of the normal neuroendocrine tissue.