Genetics of Pancreatic Neuroendocrine Tumors

Pancreatic neuroendocrine tumors (pNETs) represent a relatively rare disease; however, the incidence has been increasing during the last 2 decades. Next generation sequencing has greatly increased our understanding of driver mutations in pNETs. Sporadic pNETs have consistently presented with mutations in MEN1, DAXX/ATRX, and genes related to the mammalian target of rapamycin pathway. Inherited pNETs have traditionally been associated with multiple endocrine neoplasia type 1 , von Hippel-Lindau syndrome, neurofibromatosis type 1 , and tuberous sclerosis complex. The current review expands on the existing knowledge and the relevant updates on the genetics of pNETs.