Abstract
Pancreatic neuroendocrine tumors (PNETs) are a heterogeneous group of neoplasms that are the second most common among pancreatic neoplasms. Treatment of PNETs appears to be quite difficult because diagnosis in many patients occurs only at the latest stage when distant metastases are recognized. Therefore, treatment with drugs targeting PNET oncogenesis is a promising strategy in such patients. In this work, we review the present knowledge on the molecular nature of PNETs, and the genetic basis of PNET-associated hereditary syndromes, including multiple endocrine neoplasia type I, von Hippel-Lindau disease, neurofibromatosis type I, and tuberous sclerosis. In addition, the results of phase III, randomized, placebo-controlled trials of the efficacy of everolimus and sunitinib for treatment of extensive non-resectable PNETs are reviewed.
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