Neuroendocrine tumors (NETs) are a very heterogeneous tumor group including: (1) carcinoid gastroenteropancreatic tumors; (2) non-carcinoid gastroenteropancreatic tumors (vasoactive intestinal peptide (VIP)oma, gastrinoma, insulinoma); (3) catecholamine-secreting tumors (neuroblastoma, sympathoblastoma, ganglioneuroblastoma, ganglioneuroma, paraganglioma, phaeochromocytoma); (4) chromophobe pituitary tumors; (5) medullary carcinoma of the thyroid; (6) Merkel cell tumors; and (7) small-cell lung cancer. The gene discussed is VIP; the disease is paraganglioma.