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Pancreatic NETs: Uncommon and complex

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

The median survival for patients with well-differentiated pancreatic NETs presenting with distant metastases is 23 months.17

Most pancreatic NETs are well-differentiated, grade 1 or grade 2 tumors18,19 but can vary in clinical presentation and aggressiveness.20-22 They can be sporadic or associated with genetic syndromes,21 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).23

Although most pancreatic NETs are nonfunctional, they may be associated with symptoms and syndromes due to the oversecretion of specific hormones.20-22

Click on the graphic to launch and learn more about the different types of pancreatic NETs.

Chart showing the Classification of Neuroendocrine Tumors (NETs)