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Table 71-3

Gastrointestinal Endocrine Tumor Syndromes

SyndromeCell TypeClinical FeaturesPercentage MalignantMajor Products
Carcinoid syndromeEnterochromaffin, enterochromaffin-likeFlushing, diarrhea, wheezing, hypotension~100Serotonin, histamine, miscellaneous peptides
Zollinger-Ellison, gastrinomaNon-β islet cell, duodenal G cellPeptic ulcers, diarrhea~70Gastrin
InsulinomaIslet β cellHypoglycemia~10Insulin
VIPoma (Verner-Morrison, WDHA)Islet D1 cellDiarrhea, hypokalemia, hypochlorhydria~60Vasoactive intestinal peptide
GlucagonomaIslet A cellMild diabetes mellitus, erythema necrolytica migrans, glossitis>75Glucagon
SomatostatinomaIslet D cellDiabetes mellitus, diarrhea, steatorrhea, gallstones~70Somatostatin

Abbreviation: WDHA, watery diarrhea, hypokalemia, achlorhydria.