Pathology - Zollinger-Ellison Syndrome
Pathophysiology Acid secretion is stimulated and gastric parietal cells proliferate due to hypersecretion of the peptide hormone gastrin from a non-B-cell islet cell endocrine tumor. Gastrin mainly causes the enterochromaffin (ECL) cells of the stomach mucosa to release histamine, which in turn encourages the parietal cells to secrete acid. When the stomach pH falls below 3.0, it is generally released by enteroendocrine (G-) cells in the stomach antrum. Uncontrolled secretion of gastrin occurs when a gastrinoma other than the stomach is present, usually in the pancreas. In the small intestine, excessive acidity deactivates pancreatic enzymes and results in maldigestion, which in this instance leads to malnutrition, diarrhea, and steatorrhea. Tachycardia and low blood pressure are indicative of severe gastrointestinal bleeding. A tumor may be detected by MRI, although nuclear scintigraphy is frequently necessary. If discrete tumors are discovered, surgical resection is the best course of action. Although ZES is an uncommon cause of peptic ulcer disease, half of cases have malignant metastases to the liver and lymph nodes. ZES sufferers that also have multiple endocrine neoplasia syndrome type 1 (MEN 1) account for thirty percent of cases.
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