Zollinger-Ellison syndrome: Difference between revisions
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==Background== | ==Background== | ||
*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 [[Special:MyLanguage/peptic ulcers|peptic ulcers]] and [[Special:MyLanguage/diarrhea|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]] | |||
*Chronic [[diarrhea]] that is responsive to [[PPI]]s | *[[Special:MyLanguage/Hematemesis|Hematemesis]] | ||
*[[Gastroesophageal reflux disease]] | *Chronic [[Special:MyLanguage/diarrhea|diarrhea]] that is responsive to [[Special:MyLanguage/PPI|PPI]]s | ||
*[[Special:MyLanguage/Gastroesophageal reflux disease|Gastroesophageal reflux disease]] | |||
*Steatorrhea | *Steatorrhea | ||
*Weight loss | *Weight loss | ||
*Postprandial [[abdominal pain]] | *Postprandial [[Special:MyLanguage/abdominal pain|abdominal pain]] | ||
*[[Nausea]] | *[[Special:MyLanguage/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== | ||
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{{Abdominal Pain DDX Epigastric}} | {{Abdominal Pain DDX Epigastric}} | ||
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{{UGIB DDX}} | {{UGIB DDX}} | ||
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==Evaluation== | ==Evaluation== | ||
===Workup=== | ===Workup=== | ||
===Diagnosis=== | ===Diagnosis=== | ||
*Fasting serum gastrin concentration (>10x upper limit of normal) in the presence of gastric pH <2 is diagnostic | *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 | *If findings not diagnostic for ZES, will require secretin stimulation test | ||
==Management== | ==Management== | ||
*Proton-Pump Inhibitors | *Proton-Pump Inhibitors | ||
*Somatostatin analog (Octreotide) if no improvement | *Somatostatin analog (Octreotide) if no improvement | ||
**Direct inhibition of gastric secretion | **Direct inhibition of gastric secretion | ||
*Surgical resection | *Surgical resection | ||
==Disposition== | ==Disposition== | ||
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==References== | ==References== | ||
<references/> | <references/> | ||
[[Category:GI]] | [[Category:GI]] | ||
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Latest revision as of 00:04, 5 January 2026
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
Epigastric Pain
- Gastroesophageal reflux disease (GERD)
- Peptic ulcer disease with or without perforation
- Gastritis
- Pancreatitis
- Gallbladder disease
- Myocardial Ischemia
- Splenic Infarctionenlargement/rupture/aneurysm
- Pericarditis/Myocarditis
- Aortic dissection
- Hepatitis
- Pyelonephritis
- Pneumonia
- Pyogenic liver abscess
- Fitz-Hugh-Curtis Syndrome
- Hepatomegaly due to CHF
- Bowel obstruction
- SMA syndrome
- Pulmonary embolism
- Bezoar
- Ingested foreign body
Upper gastrointestinal bleeding
- Peptic ulcer disease (most common cause)
- Gastritis/esophagitis
- Gastric/esophageal varices
- Mallory-Weiss tear
- Malignancy
- Aortoenteric fisulta
- Boerhaave
- Dieulafoy's lesion
- Angiodysplasia
- Hemobilia
- Hemorrhagic gastritis, EtOH
- Celiac disease
- Dengue
- Other intrabdominal bleeds
- Lower GI bleeding
- Hemorrhagic pancreatitis
- Splenic rupture
- Subcapsular cavernous hemangiomas
- Peliosis hepatis
Mimics of GI Bleeding
- Hemoptysis
- Vaginal/Urethra bleeding
- ENT bleeding
- Dietary (Iron, bismuth, beets)
- Swallowed maternal blood (in neonate)
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.