Zollinger-Ellison syndrome pathophysiology: Difference between revisions
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==Pathogenesis== | ==Pathogenesis== | ||
*Zollinger-Ellison syndrome is a disorder where increased levels of [[gastrin]] are produced, causing the [[stomach]] to produce excess [[hydrochloric acid]]. Often, the cause is a [[tumor]] ([[gastrinoma]]) of the [[duodenum]] or [[pancreas]] producing the hormone [[gastrin]]. Gastrin then causes an excessive production of acid which can lead to [[peptic ulcers]] (in almost 95% of patients).<ref name="wikipedia">wikipedia.2015.https://en.wikipedia.org/wiki/Zollinger%E2%80%93Ellison_syndrome</ref> | *Zollinger-Ellison syndrome is a disorder where increased levels of [[gastrin]] are produced, causing the [[stomach]] to produce excess [[hydrochloric acid]]. Often, the cause is a [[tumor]] ([[gastrinoma]]) of the [[duodenum]] or [[pancreas]] producing the hormone [[gastrin]]. Gastrin then causes an excessive production of acid which can lead to [[peptic ulcers]] (in almost 95% of patients).<ref name="wikipedia">wikipedia.2015.https://en.wikipedia.org/wiki/Zollinger%E2%80%93Ellison_syndrome</ref> | ||
* | *Increased basal [[gastric acid]] output and [[hyperplasia|hyyperplasia]] of the fundic [[parietal cells]] occur as a result of excessive amounts of gastrin secreted by the gastrinoma tumor cells. The excessive [[gastric acid]] output overrides the mucosal defense of the gastric and duodenal wall thereby causing [[ulceration]], and inactivation of [[pancreatic]] digestive enzymes which therefore results in fat [[malabsorption]] and [[diarrhea]]. Secretory nature of [[diarrhea]] is a result of the inhibition of absorption of sodium and water by the [[small intestine]]. <ref name="pmid28722872">{{cite journal |vauthors=Cingam S, Karanchi H |title= |journal= |volume= |issue= |pages= |year= |pmid=28722872 |doi= |url=}}</ref> | ||
*The pathophysiology of ZES is | *The pathophysiology of ZES is the stimulatory action of gastrin on the parietal cells of the gastric antrum resulting in hypersecretory acid milleu. <ref name="pmid24319020">{{cite journal |vauthors=Epelboym I, Mazeh H |title=Zollinger-Ellison syndrome: classical considerations and current controversies |journal=Oncologist |volume=19 |issue=1 |pages=44–50 |year=2014 |pmid=24319020 |pmc=3903066 |doi=10.1634/theoncologist.2013-0369 |url=}}</ref> | ||
* | *Majority of patients with ZES also develop [[peptic ulcers]] which are large large and multiple in number, usually in the distal [[duodenum]] and proximal [[jejunum]] (which usually would be an uncommon location for [[ulcers]] arising due to [[Helicobacter pylori]] or by the use of [[nonsteroidal anti-inflammatory drugs]]). <ref name="pmid24319020">{{cite journal |vauthors=Epelboym I, Mazeh H |title=Zollinger-Ellison syndrome: classical considerations and current controversies |journal=Oncologist |volume=19 |issue=1 |pages=44–50 |year=2014 |pmid=24319020 |pmc=3903066 |doi=10.1634/theoncologist.2013-0369 |url=}}</ref> | ||
==Genetics== | ==Genetics== | ||
Revision as of 13:57, 27 August 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Aravind Reddy Kothagadi M.B.B.S[2] Mohamad Alkateb, MBBCh [3]
Overview
Development of Zollinger-Ellison syndrome is the result of increased levels of gastrin due to an existing gastrinoma in the duodenum or pancreas.
Pathogenesis
- Zollinger-Ellison syndrome is a disorder where increased levels of gastrin are produced, causing the stomach to produce excess hydrochloric acid. Often, the cause is a tumor (gastrinoma) of the duodenum or pancreas producing the hormone gastrin. Gastrin then causes an excessive production of acid which can lead to peptic ulcers (in almost 95% of patients).[1]
- Increased basal gastric acid output and hyyperplasia of the fundic parietal cells occur as a result of excessive amounts of gastrin secreted by the gastrinoma tumor cells. The excessive gastric acid output overrides the mucosal defense of the gastric and duodenal wall thereby causing ulceration, and inactivation of pancreatic digestive enzymes which therefore results in fat malabsorption and diarrhea. Secretory nature of diarrhea is a result of the inhibition of absorption of sodium and water by the small intestine. [2]
- The pathophysiology of ZES is the stimulatory action of gastrin on the parietal cells of the gastric antrum resulting in hypersecretory acid milleu. [3]
- Majority of patients with ZES also develop peptic ulcers which are large large and multiple in number, usually in the distal duodenum and proximal jejunum (which usually would be an uncommon location for ulcers arising due to Helicobacter pylori or by the use of nonsteroidal anti-inflammatory drugs). [3]
Genetics
- Approximately 80% of the time, the primary causative lesion is thought to arise sporadically; in the remainder of recorded cases, this entity exists as part of MEN-1, an autosomal dominant disorder characterized by tumors of the pituitary, the parathyroid, and the pancreas. [4]
Associated Conditions
Gross Pathology
- Gross pathology presents as enlarged fundic mucosal folds with cerebriform pattern.
Microscopic Pathology
- Histologically, well-differentiated neuroendocrine tumor (NET) has a typical organoid arrangement of cells with nesting, trabecular, or gyriform patterns. [2]
- The tumor cells are round with regular bland nuclei and produce large amounts of secretory granules with diffuse immunoexpression of neuroendocrine markers. In contrast, the poorly differentiated neuroendocrine tumor (NET) has atypical, sheet-like, diffuse and irregular nuclei, less cytoplasmic secretory granules, and limited biomarker immunoexpression. [2]
- An important feature for the diagnosis of neuroendocrine tumors is immunostaining for chromogranin A and synaptophysin. Gastrin immunostaining can be used to differentiate from other neuroendocrine tumors. Gastrinomas express a high density of somatostatin receptors, thus making somatostatin scintigraphy an effective localizing tool. [2]
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Well-differentiated neuroendocrine tumor of the duodenum.
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Pancreatic neuroendocrine tumour.
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Gastric neuroendocrine tumour - high magnification
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Gastric neuroendocrine tumour - intermed_magnification
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Gastric neuroendocrine tumour - low magnification
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Gastrinoma
References
- ↑ wikipedia.2015.https://en.wikipedia.org/wiki/Zollinger%E2%80%93Ellison_syndrome
- ↑ 2.0 2.1 2.2 2.3 Cingam S, Karanchi H. PMID 28722872. Missing or empty
|title=(help) - ↑ 3.0 3.1 Epelboym I, Mazeh H (2014). "Zollinger-Ellison syndrome: classical considerations and current controversies". Oncologist. 19 (1): 44–50. doi:10.1634/theoncologist.2013-0369. PMC 3903066. PMID 24319020.
- ↑ Thakker RV, Newey PJ, Walls GV, Bilezikian J, Dralle H, Ebeling PR; et al. (2012). "Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1)". J Clin Endocrinol Metab. 97 (9): 2990–3011. doi:10.1210/jc.2012-1230. PMID 22723327.