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Table 72-3

Gastrointestinal Endocrine Tumor Syndromes

SYNDROMECELL TYPECLINICAL FEATURESPERCENTAGE MALIGNANTMAJOR PRODUCTS
Carcinoid syndromeEntero-chromaffin, enterochro-maffin-likeFlushing, diarrhea, wheezing, hypotension100Serotonin, histamine, miscellaneous peptides
Zollinger-Ellison, gastrinomaNon-β islet cell, duodenal G cellPeptic ulcers, diarrhea70Gastrin
InsulinomaIslet β cellHypoglycemia10Insulin
VIPoma (Verner-Morrison, WDHA)Islet D1 cellDiarrhea, hypokalemia, hypochlorhydria60Vasoactive intestinal peptide
GlucagonomaIslet A cellMild diabetes mellitus, erythema necrolytica migrans, glossitis>75Glucagon
Somato-statinomaIslet D cellDiabetes mellitus, diarrhea, steatorrhea, gallstones70Somatostatin

Abbreviation: WDHA, watery diarrhea, hypokalemia, achlorhydria.