Treatment of metastatic disease in patients with neuroendocrine tumors.

Gastroenteropancreatic neuroendocrine tumors (GENTs) compromise a heterogeneous group of relatively uncommon neoplasms with a yearly incidence rate of 1.2 to 3.0 per 100,000 population. These tumors share numerous histologic and biologic features, allowing their consideration as a common entity. They are postulated to arise from neuroendocrine cells, but most are not from neural crest origin. Their predominant site of origin is the gastrointestinal tract, where most involve the small intestine and appendix, but are also found in the adrenal medulla, bronchopulmonary system, pancreas, thyroid, parathyroid, and paraganglia cells. A common feature is their often indolent course, but some tumors are poorly differentiated and behave aggressively. This article addresses the surgical management of endocrine malignancies and the treatment of metastatic disease in patients with neuroendocrine tumors.