Pancreatic Neuroendocrine Tumors - PNETs

Pancreatic neuroendocrine tumors (PNETs) are rare tumors of pancreatic islet cells  that account for approximately 10% of pancreatic tumors by prevalence.  Many neuroendocrine tumors arise sporadically, whereas some are associated with genetic syndromes, such as multiple endocrine neoplasia type 1 (MEN1).  PNETs can be either functional, demonstrating symptoms associated with the hypersecretion of hormones (eg, insulin, glucagon, gastrin), or nonfunctional.  Approximately 22% of patients with PNETs have functional tumors, many of which are insulinomas.  Risk for metastases varies by type; most insulinomas follow an indolent course, but other PNET types, such as gastrinomas, demonstrate a higher risk for metastases.  Laboratory testing for PNETs involves evaluation of specific biochemical markers, as determined by the clinical presentation.  Genetic testing for hereditary syndromes should be considered for all patients with PNETs. 

Tabs Content
Content Review: 
October 2019

Last Update: November 2019