Zollinger-Ellison syndrome

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Zollinger-Ellison syndrome (ZES) is a clinical syndrome that occurs secondary to a gastrinoma.

Clinical presentation

Diagnosis inof ZES is often delayed by 5-7 years after onset of symptoms ².

Pathology

Gastrinomas are usually multiple and typically located in the pancreas or duodenum. These~~carcinoid tumours~~ 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)

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

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

-<p><strong>Zollinger-Ellison</strong> <strong>syndrome</strong> (<strong>ZES</strong>) is a clinical syndrome that occurs secondary to a <a title="Gastrinoma" href="/articles/gastrinoma">gastrinoma</a>. </p><h4>Clinical presentation</h4><p>Diagnosis in ZES is often delayed by 5-7 years after onset of symptoms <sup>2</sup>. </p><h4>Pathology</h4><p>Gastrinomas are usually multiple and typically located in the pancreas or duodenum. These <a title="Carcinoid tumour" href="/articles/carcinoid-tumours-1">carcinoid tumours</a> 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 <sup>1</sup>. </p><h5>Markers</h5><ul><li>increased gastrin levels in fasting patients (but not specific) </li></ul><h5>Associations</h5><ul><li>
-<a title="Multiple endocrine neoplasia type 1 (MEN I)" href="/articles/multiple-endocrine-neoplasia-type-i-1">multiple endocrine neoplasia (MEN) type 1:</a> ZES occurs when gastrinoma is functional</li></ul><h4>Radiographic features</h4><h5>Fluoroscopy</h5><p>On double-contrast upper gastrointestinal studies the following features may be seen <sup>4, 5</sup>:</p><ul>
+<p><strong>Zollinger-Ellison</strong> <strong>syndrome</strong> (<strong>ZES</strong>) is a clinical syndrome that occurs secondary to a <a href="/articles/gastrinoma">gastrinoma</a>. </p><h4>Clinical presentation</h4><p>Diagnosis of ZES is often delayed by 5-7 years after onset of symptoms <sup>2</sup>. </p><h4>Pathology</h4><p>Gastrinomas are usually multiple and typically located in the pancreas or duodenum. These tumours secrete gastrin that result in hypersecretion of gastric acid, which in turn results in diarrhoea, gastritis, severe <a href="/articles/gastro-oesophageal-reflux-disease">gastro-oesophageal reflux disease</a> and <a href="/articles/peptic-ulcer-disease">peptic ulcer disease</a> <sup>1</sup>. </p><h5>Markers</h5><ul><li>increased gastrin levels in fasting patients (but not specific) </li></ul><h5>Associations</h5><ul><li>
+<a href="/articles/multiple-endocrine-neoplasia-type-i-1">multiple endocrine neoplasia (MEN) type 1:</a> ZES occurs when gastrinoma is functional</li></ul><h4>Radiographic features</h4><h5>Fluoroscopy</h5><p>On double-contrast upper gastrointestinal studies the following features may be seen <sup>4, 5</sup>:</p><ul>
~~-<li>barium may be diluted by high volume of fluid in the <a title="Stomach" href="/articles/stomach">stomach</a>~~
+<li>barium may be diluted by high volume of fluid in the <a href="/articles/stomach">stomach</a>
~~-<a title="gastritis" href="/articles/gastritis">gastritis</a> from other causes, e.g. <em>H. pylori</em> infection, hypertrophic gastritis</li>~~
+<a href="/articles/gastritis">gastritis</a> from other causes, e.g. <em>H. pylori</em> infection, hypertrophic gastritis</li>
~~-<a title="Gastric lymphoma" href="/articles/gastric-lymphoma">gastric lymphoma</a> <sup>5</sup>~~
+<a href="/articles/gastric-lymphoma">gastric lymphoma</a> <sup>5</sup>

References changed:

3. Metz DC, Jensen RT. Gastrointestinal neuroendocrine tumors: pancreatic endocrine tumors. Gastroenterology. 2008;135 (5): 1469-92. <a href="http://dx.doi.org/10.1053/j.gastro.2008.05.047">doi:10.1053/j.gastro.2008.05.047</a> - <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2612755">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/18703061">Pubmed citation</a><span class="auto"></span>
4. Levy AD, Sobin LH. From the archives of the AFIP: Gastrointestinal carcinoids: imaging features with clinicopathologic comparison. Radiographics. 2007;27 (1): 237-57. <a href="http://radiographics.rsna.org/content/27/1/237.full">Radiographics (full text)</a> - <a href="http://dx.doi.org/10.1148/rg.271065169">doi:10.1148/rg.271065169</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/17235010">Pubmed citation</a><span class="ref_v3"></span>
5. Gore RM, Levine MS. High Yield Imaging: Gastrointestinal: Expert Consult - Online and Print, 1e (HIGH YIELD in Radiology). Saunders. ISBN:1416055444. <a href="http://books.google.com/books?vid=ISBN1416055444">Read it at Google Books</a> - <a href="http://www.amazon.com/gp/product/1416055444">Find it at Amazon</a><span class="auto"></span>
1. Ito T, Cadiot G, Jensen RT. Diagnosis of Zollinger-Ellison syndrome: increasingly difficult. World J. Gastroenterol. 2012;18 (39): 5495-503. <a href="http://dx.doi.org/10.3748/wjg.v18.i39.5495">doi:10.3748/wjg.v18.i39.5495</a> - <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3482635">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/23112541">Pubmed citation</a><span class="auto"></span>
2. Plöckinger U. Diagnosis and Treatment of Gastrinomas in Multiple Endocrine Neoplasia Type 1 (MEN-1). Cancers (Basel). 2012;4 (4): 39-54. <a href="http://dx.doi.org/10.3390/cancers4010039">doi:10.3390/cancers4010039</a> - <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3712689">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/24213225">Pubmed citation</a><span class="auto"></span>

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