Table 1:
Pancreatic Neuroendocrine Tumors
Tumor Type
%A
Secretory Product
Clinical Features
Laboratory Testing
Symptomatic Treatment
Insulinoma
40-60
Insulin
  • Hypoglycemia
  • Symptoms of catecholamine excess
  • 90% benign
  • Insulin level/
    C-peptide
  • 72-hour inpatient fast with monitoring of glucose and insulin levels
  • Dietary measures
  • Octreotide
  • Diazoxide
Gastrinoma
20-50
Gastrin
  • Peptic ulcer disease
  • GERD
  • Secretory diarrhea
  • Most common NET in MEN 1
  • 60% to 90% malignant
  • Fasting serum gastrin
  • Gastric pH analysis
  • Gastrin provocation testing (calcium or secretin challenge)
  • Proton pump inhibitor
  • Octreotide
Glucagonoma
Rare
Glucagon
  • Glucose intolerance
  • Migratory necrolytic erythema
  • Weight loss
  • Anemia
  • 90% malignant
  • Serum glucagon
  • Octreotide
  • Insulin
  • Zinc supplement (rash)
  • TPN (malnutrition)
Somatostatinoma
Rare
Somatostatin
  • Diabetes
  • Gallstones
  • Secretory diarrhea
  • Clinical and pathologic diagnosis
  • Increased somatostatin-like immunoreactivity in resected tumor
  • Octreotide
VIPoma
Rare
Vasoactive intestinal peptide
  • Cholera-like, secretory diarrhea
  • Hypokalemia
  • Hypochlorhydria
  • Serum VIP
  • Octreotide
APercent among neuroendocrine tumors, GERD = gastroesophageal reflux disease; MEN 1 = multiple endocrine neoplasia type 1; NET = neuroendocrine tumor; TPN = total parenteral nutrition; VIP = vasoactive intestinal peptide
Copyright 2005 The Cleveland Clinic Foundation

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