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Zollinger-Ellison syndrome (ZES) is a clinical syndrome that occurs secondary to elevated gastrin levels produced by a gastrinoma.
Diagnosis of Zollinger-Ellison syndrome is often delayed by 5-7 years after the onset of symptoms 2.
Gastrinomas are usually multiple and typically located in the duodenum (more common) or pancreas (less common). These tumors secrete gastrin that results in hypersecretion of gastric acid, which in turn results in diarrhea, gastritis, severe gastro-esophageal reflux disease and peptic ulcer disease 1.
increased gastrin levels in fasting patients (but not specific, and some data suggest that clinical assays may be unreliable 6)
multiple endocrine neoplasia (MEN) type 1: Zollinger-Ellison syndrome occurs when the gastrinoma is functional
On double-contrast upper gastrointestinal studies the following features may be seen 4,5:
thickened rugal folds
multinodular gastric contour
erosions and ulcers, especially in atypical locations
barium may be diluted by the high volume of fluid in the stomach
negative contrast may be used to distend the stomach
thickened rugal folds
multiple gastric nodules/masses 4
Lesions show increased uptake on somatostatin receptor scintigraphy using 111In-labeled octreotide and somatostatin receptor PET/CT using 68Ga-DOTANOC 9.
Treatment and prognosis
Surgery plays a vital role 8. Death from complications of Zollinger-Ellison syndrome (e.g. perforation, hemorrhage) can occur.
History and etymology
It is named after Robert M Zollinger and Edwin H Ellison, who in April 1956 described two cases of severe, multifocal ulcerative lesions of the proximal gastrointestinal tract, which were remittent, refractory to surgery, and associated with tumors in the adjacent pancreas 7.
Possible differential considerations include:
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- 7. Zollinger RM, Ellison EH. Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann. Surg. 2003;142 (4): 709-23. Free text at pubmed - Pubmed citation
- 8. Epelboym I, Mazeh H. Zollinger-Ellison syndrome: classical considerations and current controversies. Oncologist. 2014;19 (1): 44-50. doi:10.1634/theoncologist.2013-0369 - Free text at pubmed - Pubmed citation
- 9. Naswa N, Sharma P, Soundararajan R et al. Diagnostic Performance of Somatostatin Receptor PET/CT Using 68Ga-DOTANOC in Gastrinoma Patients with Negative or Equivocal CT Findings. Abdom Imaging. 2012;38(3):552-60. doi:10.1007/s00261-012-9925-z - Pubmed