Zollinger-Ellison syndrome is a condition caused by gastrin-producing neuroendocrine tumor that causes gastric acid hypersecretion and peptic ulcer disease.
Can be sporadic or associated with multiple endocrine neoplasia syndrome type 1.
Common presentation includes refractory peptic ulcer disease, abdominal pain, diarrhea, and gastroesophageal disease.
The main goal of treatment is control of gastric hypersecretion with proton-pump inhibitors.
The most common cause of morbidity and mortality is metastatic gastrinoma.
Zollinger-Ellison syndrome (ZES) is a condition caused by a gastrin-secreting tumor that causes hypersecretion of gastric acid leading to ulcer disease. It most commonly presents with abdominal pain (70% to 100%), diarrhea (37% to 73%), and GERD (44%). Less common presentations include weight loss, gastrointestinal bleeding, nausea, and vomiting.
History and exam
Key diagnostic factors
- refractory or recurrent peptic ulcer disease
Other diagnostic factors
- epigastric abdominal pain
- gastroesophageal reflux disease
- gastrointestinal bleed
- bone pain
- multiple endocrine neoplasia (MEN) type 1
1st investigations to order
- fasting serum gastrin
- acid output (basal and maximal)
- secretin infusion test
- calcium infusion test
Investigations to consider
- upper gastrointestinal endoscopy
- endoscopic ultrasound (EUS)
- MRI liver
- spiral CT liver
- somatostatin receptor scintigraphy (SRS)
- gallium(Ga)-68 DOTATATE
- glucagon test
- serum ghrelin
localized disease: sporadic
localized disease: MEN1 syndrome
metastatic disease: predominantly hepatic
metastatic disease: extrahepatic
- Atrophic gastritis
- Peptic ulcer disease from Helicobacter pylori infection
- Idiopathic gastric hypersecretion
- Informed consent for GI endoscopic procedures
- Adverse events associated with EUS and EUS-guided procedures
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