The endocrine cells of the pancreas are formed into clusters called islets of Langerhans, which make up about 1% to 2% of the total mass of the pancreas.11 The islets of Langerhans produce 3 important hormones: insulin, glucagon, and somatostatin.12 Pancreatic neuroendocrine tumors (NETs) arise from these cells.13
Pancreatic NETs are a distinct, much less common form of pancreatic cancer and have notably different tumor biology from pancreatic adenocarcinomas.14-16
Most pancreatic NETs are well-differentiated, grade 1 or grade 2 tumors18,19 but can vary in clinical presentation and aggressiveness.8,20,21 They can be sporadic or associated with genetic syndromes,20 such as multiple endocrine neoplasia type 1 (MEN-1), von Hippel-Lindau syndrome (VHL), von Recklinghausen disease (neurofibromatosis [NF-1]), and tuberous sclerosis complex (TSC).22
Although most pancreatic NETs are nonfunctional, they may be associated with symptoms and syndromes due to the oversecretion of specific hormones.8,20,21
Click on the graphic to launch and learn more about the different types of pancreatic NETs.