Pancreatic neuroendocrine tumors: targeting the molecular basis of disease.

PURPOSE OF REVIEW: Pancreatic neuroendocrine tumors (pNETs) are a rare and heterogeneous group of neoplasia. Presentation of these tumors can vary widely. Current treatment modalities range from potentially curative surgical interventions in localized disease to the use of varied hormonal analogues, cytotoxic agents and targeted therapy for the management of locally advanced and metastatic disease. With such a wide variety of therapeutic modalities, clinicians are faced with the task of building an effective and comprehensive treatment strategy for their patients.

RECENT FINDINGS: Targeted therapy for pNET is limited to sunitinib and everolimus. There have been a number of important studies assessing the efficacy of other targeted agents, in addition to the conjugation of these agents in the management of advanced pNET. This review will stand to highlight currently available targeted therapies for the treatment of advanced pNET.

SUMMARY: The use of targeted agents in the management of advanced pNET has significant potential to change the current standard of care. In addition to the use of long-acting somatostatin analogues, targeting the mammalian target of rapamycin and vascular endothelial growth factor pathways can be well tolerated and may lead to long periods of disease control in a wide variety of neuroendocrine tumors involving the pancreas.