Clinical
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
