Zollinger-Ellison syndrome
Updates to Article Attributes
Zollinger-Ellison syndrome (ZES) is a clinical syndrome that occurs secondary to a gastrinoma.
Clinical presentation
Diagnosis of ZES is often delayed by 5-7 years after onset of symptoms 2.
Pathology
Gastrinomas are usually multiple and typically located in the duodenum (more common) or pancreas (less common). These tumours secrete gastrin that result in hypersecretion of gastric acid, which in turn results in diarrhoea, gastritis, severe gastro-oesophageal reflux disease and peptic ulcer disease 1.
Markers
- increased gastrin levels in fasting patients (but not specific, and some data suggest that clinical assays may be unreliable 6)
Associations
- multiple endocrine neoplasia (MEN) type 1: ZES occurs when gastrinoma is functional
Radiographic features
Fluoroscopy
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 high volume of fluid in the stomach
CT
- negative contrast may be used to distend the stomach
- thickened rugal folds
- multiple gastric nodules/masses 4
Treatment and prognosis
DeathSurgery plays a key role 8. Death from complications of ZES (e.g. perforation, haemorrhage) can occur.
Differential diagnosis
- gastritis from other causes, e.g. H. pylori infection, hypertrophic gastritis
- gastric lymphoma 5
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, that were remittent, refractory to surgery and associated with tumors in the adjacent pancreas 7.
-</ul><h4>Treatment and prognosis</h4><p>Death from complications of ZES (e.g. perforation, haemorrhage) can occur. </p><h4>Differential diagnosis</h4><ul>- +</ul><h4>Treatment and prognosis</h4><p>Surgery plays a key role <sup>8</sup>. Death from complications of ZES (e.g. perforation, haemorrhage) can occur. </p><h4>Differential diagnosis</h4><ul>
-</ul>- +</ul><h4>Etymology</h4><p>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, that were remittent, refractory to surgery and associated with tumors in the adjacent pancreas <sup>7</sup>. </p><p> </p>
References changed:
- 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. <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1465210">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/13259432">Pubmed citation</a><span class="auto"></span>
- 8. Epelboym I, Mazeh H. Zollinger-Ellison syndrome: classical considerations and current controversies. Oncologist. 2014;19 (1): 44-50. <a href="http://dx.doi.org/10.1634/theoncologist.2013-0369">doi:10.1634/theoncologist.2013-0369</a> - <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3903066">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/24319020">Pubmed citation</a><span class="auto"></span>
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