Neuroendocrine tumors of the pancreas

Purpose of review Pancreatic neuroendocrine tumors (PNETs) are rare tumors, with an incidence of one per 100 000 individuals per year, and they account for 1-2% of all pancreatic neoplasms. PNETs are a heterogeneous group with varying clinical presentation, tumor biology and prognosis. This article reviews the current diagnostic strategy and treatment armamentarium for PNETs. Special attention is paid to recent and ongoing developments in treatment, particularly with regards to multimodality treatment and newer systemic therapies for unresectable disease. Recent findings There has been significant progress in the genetic understanding of hereditary syndromes in regards to PNETs, as well as in the diagnosis and treatment of resectable and nonresectable PNETs. Whereas surgical therapy remains the most advisable therapy for resectable neuroendocrine tumors of the pancreas, there have been significant recent advances in systemic therapy for those with unresectable disease. Results from recent clinical trials examining mammalian target of rapamycin inhibitors and tyrosine kinase inhibitors for unresectable disease are promising in expanding treatment options for metastatic PNETs. Summary Neuroendocrine tumors of the pancreas are a heterogeneous group of tumors with varying clinical presentation, tumor biology and prognosis. Clinicians must be aware of the variety of manifestations of this disease, as well as the role of systemic chemotherapy in treatment of unresectable disease.