• These names depend on the functional status of the tumor
  • Defined by clinical plus serologic findings and not by immunohistochemistry
  • Except for PPoma (see below)
  • Neoplasms that are positive for a peptide product by immunohistochemistry but lack the corresponding clinical syndrome are designated beta-cell neoplasms, alpha-cell neoplasms etc.
• Insulinoma
  • 30-40% of functional tumors
  • Elevated serum insulin, pro-insulin and C-peptide
  • Symptoms of hypoglycemia w/ relief after glucose administration
  • Most insulinomas are benign, but between 5-10% associated with metastasis
• Gastrinoma
  • 12.5% of functional tumors
  • 80% present with duodenal ulcer (Zollinger-Ellison syndrome)
  • Serum gastrin levels >1000 pg/mL are diagnostic
  • Between 30-50% have metastatic disease at presentation
    • Can metastasize while still very small
  • 50% 10 year survival
  • May cause secondary gastric endocrine cell hyperplasia and neoplasia
  • Most gastrinomas are extra-pancreatic (duodenal) in origin
• Glucagonoma
  • 8-13% of functional tumors
  • Necrolytic migratory erythema (70%), stomatitis, weight loss, diabetes mellitus (50%)
  • Elevated fasting plasma glucagon
  • Between 60-70% have metastatic disease at presentation
• VIPoma
  • 10% of functional tumors
  • Watery diarrhea with hypokalemia and achlorhydria (Verner-Morrison syndrome, pancreatic cholera)
  • VIP may also be secreted by neurogenic neoplasms
  • 60% 5 year survival
• Somatostatinoma
  • 2-3% of pancreatic endocrine neoplasms
  • Diabetes mellitus, hypochlorhydria, cholelithiasis, diarrhea, anemia, weight loss
  • 50% 10 year mortality
• Pancreatic polypeptide cell-oma (PPoma)
  • No clinical syndrome
  • Recognized by immunohistochemistry (>50% of cells positive)
  • Elevated serum PP is not sufficient as it is commonly elevated with other peptides
  • 44% metastatic rate
• Serotonin secreting pancreatic endocrine neoplasm
  • Alternate name: pancreatic carcinoid tumor
  • Rare
  • Carcinoid syndrome
    • Flushing, diarrhea, bronchoconstriction
    • Usually present only if metastatic to liver
  • Elevated serum 5-HT and/or urine 5-HIAA
  • Extra-pancreatic primary must be ruled out
• Rare neoplasms secrete other hormones
  • ACTH, parathyroid hormone, growth hormone, calcitonin
• Multiple endocrine neoplasia 1 (MEN1) syndrome
  • Over half have a pancreatic endocrine neoplasm
    • Microadenomas are frequent and multiple
    • Most common types are gastrinoma, insulinomas and PPoma
    • Rare types are VIPoma and glucagonoma
• von Hippel Lindau syndrome
  • 5-10% have a pancreatic endocrine neoplasm
    • May show clear cell change
    • Most nonfunctional