Clinical features of functional pancreatic neuroendocrine tumors

Name	Biologically active peptide(s)	Incidence (new cases/10⁶ population/year)	Tumor location	Most common symptoms/signs
Most common syndromes
Insulinoma	Insulin	1 to 3	Pancreas (>99%)	Hypoglycemic syndromes (Whipple's triad)
Zollinger-Ellison syndrome	Gastrin	0.5 to 2	Duodenum (70%) Pancreas (25%) Other sites (5%)	Abdominal pain, gastroesophageal reflux, diarrhea, duodenal ulcers, PUD/GERD
Less common syndromes (additional, rarer syndromes also exist)
VIPoma (Verner-Morrison syndrome, pancreatic cholera, WDHA syndrome)	Vasoactive intestinal peptide	0.05 to 0.2	Pancreas (90%, adult) Other (10%, neural, adrenal, periganglionic)	Diarrhea, hypokalemia, dehydration
Glucagonoma	Glucagon	0.01 to 0.1	Pancreas (100%)	Rash, glucose intolerance, necrolytic migratory erythema, weight loss
Somatostatinoma	Somatostatin	Rare	Pancreas (55%) Duodenum/jejunum (44%)	Diabetes mellitus, cholelithiasis, diarrhea
ACTHoma/Cushing's syndrome	ACTH	Rare	Pancreas (4 to 16% all ectopic Cushing's)	Cushing's syndrome
Pancreatic NET causing carcinoid syndrome	Serotonin	Rare	Pancreas (<1% all carcinoid syndrome)	Flushing, diarrhea
PTHrp-oma (hypercalcemia)	PTHrp, others unknown	Rare	Pancreas	Symptoms due to hypercalcemia (mimics primary hyperparathyroidism)

PUD: peptic ulcer disease; GERD: gastroesophageal reflux disease; WDHA: watery diarrhea, hypokalemia, and achlorhydria; NET: neuroendocrine tumor; PTHrp: parathyroid hormone-related protein.

Adapted with permission from: Jensen RT, Cadiot G, Brandi ML, et al. ENETS Consensus Guidelines for the management of patients with digestive neuroendocrine neoplasms: functional pancreatic endocrine tumor syndromes. Neuroendocrinology 2012; 95:98. Copyright © 2012 S. Karger AG, Basel.

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Clinical features of functional pancreatic neuroendocrine tumors