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Keywords:

  • pancreatic neuroendocrine tumor;
  • classification;
  • targeted therapy

Abstract

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.