Zollinger-Ellison syndrome: Difference between revisions

 
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*Also known as "S-E syndrome"
*Also known as "S-E syndrome"
*Caused by secretion of gastrin by duodenal/pancreatic neuroendocrine tumors (gastrinomas) —> high gastric acid output by parietal cells
*Caused by secretion of gastrin by duodenal/pancreatic neuroendocrine tumors (gastrinomas) —> high gastric acid output by parietal cells
*Associated with peptic ulcers and diarrhea
*Associated with [[peptic ulcers]] and [[diarrhea]]
*60-90% malignant <ref> 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.</ref>, only 20% resectable; 25% of gastrinoma patients have multiple endocrine neoplasia type I; >80% of gastrinomas found in gastrinoma triangle.
*60-90% malignant <ref> 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.</ref>, only 20% resectable; 25% of gastrinoma patients have multiple endocrine neoplasia type I; >80% of gastrinomas found in gastrinoma triangle.


==Clinical Features==
==Clinical Features==
*Hematemesis
*[[Hematemesis]]
*Chronic diarrhea that is responsive to PPIs
*Chronic [[diarrhea]] that is responsive to [[PPI]]s
*[[Gastroesophageal reflux disease]]
*[[Gastroesophageal reflux disease]]
*Steatorrhea
*Steatorrhea
*Weight loss
*Weight loss
*Postprandial abdominal pain  
*Postprandial [[abdominal pain]]
*Nausea
*[[Nausea]]
*Wheezes on auscultation
*Wheezes on auscultation
*Evidence of malnourishment and decreased oral intake
*Evidence of malnourishment and decreased oral intake


==Differential Diagnosis==
==Differential Diagnosis==
 
{{Abdominal Pain DDX Epigastric}}
{{UGIB DDX}}


==Evaluation==
==Evaluation==

Latest revision as of 23:37, 7 December 2022

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

Differential Diagnosis

Epigastric Pain

Upper gastrointestinal bleeding

Mimics of GI Bleeding

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

  1. 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.