Systemic Therapies for Advanced Pancreatic Neuroendocrine Tumors.

Pancreatic neuroendocrine tumors are an uncommon tumor type and compose 1% to 2% of all pancreatic neoplasms. They are rarely localized at presentation and are typically diagnosed in the presence of metastatic disease. The management poses a significant challenge because of the heterogeneous clinical presentations and varying degrees of aggressiveness. A variety of systemic therapies have been developed for the management of pancreatic neuroendocrine tumors, including somatostatin analogues, a select group of cytotoxic chemotherapy agents, and targeted or biological agents. This article reviews the available systemic therapy options for advanced pancreatic neuroendocrine tumors.