Somatostatinoma, Glucagonoma, insulinoma, vipoma, gastrinoma

Pancreas NeoEndocrine/Islet cell Tumors

Sometimes associated with MEN I (multiple endocrine neoplasia)

Somatostatinoma

  • tumor of the delta cells of the endocrine pancreas
  • triad of: Diabetes Mellitus, Steatorrhea, and Gallstones
  • Most are malignant and have metastasized

Glucagonoma

  • Necrolytic Migratory Erythema (NME). ¬†found in 70%
  • Diabetes Mellitus
  • Most are malignant and have metastasized

VIPOMA

  • Vasoactive Intestinal Polypeptide tumor
  • Verner Morrison syndrome
  • Causes WDHHA: ¬†Watery Diarrhea, Hypokalemia, Hypochlorhydria, Achlorhydria
  • Activation of cellular adenylate cyclase and cAMP production
  • 50% malignant

Insulinoma

  • 10% malignant
  • Whipple’s triad of pancreatic insulinoma – Hypoglycemia, symptoms of hypoglycemia, relief of symptoms when glucose is given.

Gastrinoma

  • Zollinger-Ellison Syndrome
  • Excess gastric acid production
  • Can arise in the duodenum or pancreas
  • 60% malignant