Advances in the systemic treatment of pancreatic neuroendocrine tumors.

Constituting about 1-2% of all tumors of the pancreas, pancreatic neuroendocrine tumors (PNETs) are a subgroup of gastroenetropancreatic neuroendocrine tumors (GEP-NETs) with distinct tumor genetics, biology, and clinicopathological features. Surgical resection is amenable only in a minority of the cases so systemic therapies are considered in most of them. The goals of medical treatment are to control the associated symptoms and signs of the specific tumors and to shrink the tumor mass. Somatostatin analogues can, not only decrease the secretion of peptides and inhibit their functions but also stop tumor growth. Other medical options for limiting tumor growth include interferon, systemic chemotherapy, and targeted therapies including, angiogenesis inhibitors, epidermal growth factor inhibitors, and mTOR inhibitors. Newer agents are tested and the treatment options expected to increase in the near future. Meanwhile optimal use of the available therapeutic strategies is critical.