Table I. Pancreatic Neuroendocrine Tumors (Pnets)

Table I. Pancreatic neuroendocrine tumors (PNETs)

Name of tumor Name of Main signs or Location Malignancy (%) Hormone causing syndrome symptoms (%) syndrome I. Functional PNET Gastrinoma Zollinger-Ellison Abdominal pain, Pancreas – 30% 60-90% Gastrin syndrome diarrhea, GERD Duodenum – 60 symptoms Other – 10 Insulinoma Insulinoma Hypoglycemic Pancreas – 100 5-15 Insulin symptoms Glucagonoma Glucagonoma Dermatitis, Pancreas – 100 60 Glucagon diabetes/glucose intolerance, weight loss

VIPoma VernerMorrison Severe water Pancreas – 90 80 Vasoactive intestinal Pancreatic diarrhea, Other – 10 (neural, peptide (VIP) cholera hypokalemia adrenal, periganglionic WDHA tissue) Somatostatinoma Somatostatinoma Diabetes mellitus, Pancreas – 56 60 Somatostatin cholelithiasis, Duodenum/jejunum − 44 diarrhea

GRFoma GRFoma Acromegaly Pancreas – 30 30 Growth hormone- Lung – 54 releasing factor Jejunum – 7 Other – 13 (adrenal, foregut, retroperitoneum) ACTHoma ACTHoma Cushing’s Pancreas 4-16 of all, >95 Adrenocorticotropic syndrome ectopic Cushing’s hormone (ACTH)

PNET causing PNET causing Diarrhea, flushing Pancreas − <1% of all 60-90 Serotonin, Tachykinins carcinoid syndrome carcinoid carcinoids syndrome PNET causing PNET causing Signs/symptoms of Pancreas – (rare cause of >85 PTHrP, other unknown hypercalcemia hypercalcemia hypercalcemia hypercalcemia) II. Nonfunctional PNET Nonfunctioning Nonfunctional Weight loss, Pancreas – 100 60-90 None: pancreatic PPoma abdominal mass, polypeptide (PPoma) hepatomegaly, chromogranin released but no symptoms due to their hypersecretion

Abbreviations: PNET = pancreatic neuroendocrine tumor; WDHA = Watery diarrhea, hypokalemia, and achlorhydria; PP = pancreatic polypeptide; PTHrP = parathormone-related peptide; GRF = growth hormone-releasing factor.