Gastrointestinal Endocrine Tumor Syndromes
Syndrome | Cell Type | Clinical Features | Percentage Malignant | Major Products |
---|---|---|---|---|
Carcinoid syndrome | Enterochromaffin, enterochromaffin-like | Flushing, diarrhea, wheezing, hypotension | ~100 | Serotonin, histamine, miscellaneous peptides |
Zollinger-Ellison, gastrinoma | Non-β islet cell, duodenal G cell | Peptic ulcers, diarrhea | ~70 | Gastrin |
Insulinoma | Islet β cell | Hypoglycemia | ~10 | Insulin |
VIPoma (Verner-Morrison, WDHA) | Islet D1 cell | Diarrhea, hypokalemia, hypochlorhydria | ~60 | Vasoactive intestinal peptide |
Glucagonoma | Islet A cell | Mild diabetes mellitus, erythema necrolytica migrans, glossitis | >75 | Glucagon |
Somatostatinoma | Islet D cell | Diabetes mellitus, diarrhea, steatorrhea, gallstones | ~70 | Somatostatin |
Abbreviation: WDHA, watery diarrhea, hypokalemia, achlorhydria.