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Int J Cancer. 2012 Sep 1;131(5):1013-22. doi: 10.1002/ijc.27543. Epub 2012 Apr 16.

Pancreatic neuroendocrine tumors: a comprehensive review.

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Department of Surgery, Shanghai Institute of Digestive Surgery, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, People's Republic of China.


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 (SUTENTĀ®, Pfizer Inc, NYC) and everolimus (AFINITORĀ®, 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. SUTENTĀ® 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.

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