Pancreatic neuroendocrine tumors: A comprehensive review

Pancreatic neuroendocrine tumors (NETs) are a heterogeneous group of tumors. Despite being relatively rare, representing just 1–2% of all pancreatic neoplasms, the incidence of pancreatic NET has increased over the past two decades. Although the primary treatment for localized NET is surgical resection, there is still a lack of effective therapeutic options for patients with advanced unresectable pancreatic NET. Recently, the targeted agents sunitinib malate (SUTENT®, Pfizer Inc, NYC) and everolimus (AFINITOR®, Novartis, Basel, Switzerland)—both with different mechanisms of action—received United States Food and Drug Administration approval for the treatment of progressive, well‐differentiated, pancreatic NET in patients with unresectable, locally advanced or metastatic disease. SUTENT® also received approval for this indication by the European Commission in 2010. Our article presents an overview of pancreatic NET, with a focus on their diagnostic work‐up, clinical presentation and treatment options. Topics for further investigation of targeted therapy are also discussed.