Zollinger-Ellison syndrome
Background
- Also known as "S-E syndrome"
- Caused by secretion of gastrin by duodenal/pancreatic neuroendocrine tumors (gastrinomas) —> high gastric acid output by parietal cells
- Associated with peptic ulcers and diarrhea
- 60-90% malignant [1], only 20% resectable; 25% of gastrinoma patients have multiple endocrine neoplasia type I; >80% of gastrinomas found in gastrinoma triangle.
Clinical Features
- Hematemesis
- Chronic diarrhea that is responsive to PPIs
- Gastroesophageal reflux disease
- Steatorrhea
- Weight loss
- Postprandial abdominal pain
- Nausea
- Wheezes on auscultation
- Evidence of malnourishment and decreased oral intake
Differential Diagnosis
Evaluation
Workup
Diagnosis
- Fasting serum gastrin concentration (>10x upper limit of normal) in the presence of gastric pH <2 is diagnostic
- If findings not diagnostic for ZES, will require secretin stimulation test
Management
- Proton-Pump Inhibitors
- Somatostatin analog (Octreotide) if no improvement
- Direct inhibition of gastric secretion
- Surgical resection
Disposition
See Also
External Links
References
- ↑ Roy PK, Venzon DJ, Shojamanesh H, Abou-Saif A, Peghini P, Doppman JL, Gibril F, Jensen RT. Zollinger-Ellison syndrome. Clinical presentation in 261 patients. Medicine (Baltimore). 2000 Nov;79(6):379-411. doi: 10.1097/00005792-200011000-00004. PMID: 11144036.