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
Joseph R. Pisegna, MD
Professor of Medicine
Division of Gastroenterology, Hepatology, and Parenteral Nutrition
David Geffen School of Medicine at University of California
VA Greater Los Angeles Healthcare System
Department of Veterans Affairs
JRP is an author of a number of references cited in this topic.
Dr Joseph R. Pisegna would like to gratefully acknowledge Dr Deepinder Goyal, Dr Jihane Benhammou, Dr David Oh, and Dr Hank Wang, previous contributors to this topic. DG, JB, DO, and HW declare that they have no competing interests.
Peter Draganov, MD
Associate Professor of Medicine Division
Gastroenterology, Hepatology and Nutrition
University of Florida
PD declares that he has no competing interests.
Petros Perros, BSc, MBBS, MD, FRCP
Newcastle-Upon-Tyne Hospitals Trust
PP declares that he has no competing interests.
- 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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