Pheochromocytoma causes: Difference between revisions
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Familial pheochromocytoma may be caused by a mutation of either ''[[VHL]]'', ''RET'', ''NF1'', ''[[SDHB]]'' or ''[[SDHD]]'' genes. | Familial pheochromocytoma may be caused by a mutation of either ''[[VHL]]'', ''RET'', ''NF1'', ''[[SDHB]]'' or ''[[SDHD]]'' genes. | ||
==Causes== | ==Causes== | ||
* Pheochromocytoma develops in called [[chromaffin cells]], found in | * Pheochromocytoma develops in called [[chromaffin cells]], found in the [[adrenal medulla]]. | ||
* Chromaffin cells typically secrete adrenaline, [[Norepinephrine|noradrenaline]] and [[dopamine]]. | * [[Chromaffin cells]] typically secrete [[adrenaline]], [[Norepinephrine|noradrenaline]], and [[dopamine]]. | ||
* These hormones are sympathetic stimulants. | * These hormones are sympathetic stimulants. | ||
* Pheochromocytoma results in the irregular and excessive release of these hormones causing hypertension and tachycardia. Approximately 10% are located in chromaffin tissue outside of the adrenal gland, The most common extra-adrenal locations are the abdomen and thorax | * Pheochromocytoma results in the irregular and excessive release of these hormones causing hypertension and [[Tachycardia|tachycardia.]] Approximately 10% are located in [[Chromaffin cell|chromaffin tissue]] outside of the [[adrenal gland]], The most common extra-adrenal locations are the abdomen and [[thorax]] | ||
* Approximately 10% of tumors are malignant. Commonest sites of spread are | * Approximately 10% of tumors are malignant. Commonest sites of spread are the base of skull, head, and neck. | ||
* | * The genetic base of pheochromocytoma depends on 2 clusters: cluster 1 tumors are [[Norepinephrine|noradrenergic]]. Cluster 2 tumors are [[adrenergic]].<ref name="pmid23933153">{{cite journal| author=King KS, Pacak K| title=Familial pheochromocytomas and paragangliomas. | journal=Mol Cell Endocrinol | year= 2014 | volume= 386 | issue= 1-2 | pages= 92-100 | pmid=23933153 | doi=10.1016/j.mce.2013.07.032 | pmc=3917973 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23933153 }}</ref> | ||
{| class="wikitable" | {| class="wikitable" | ||
!Cluster 1 | !Cluster 1 | ||
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|- | |- | ||
! | ! | ||
* Succinate dehydrogenase (SDH) subunit genes | * [[Succinate dehydrogenase]] (SDH) subunit genes | ||
* [[Von Hippel-Lindau Disease|Von Hippel-Lindau (VHL) disease]] | * [[Von Hippel-Lindau Disease|Von Hippel-Lindau (VHL) disease]] | ||
* Fumarate hydratase gene mutations | * [[Fumarate hydratase|Fumarate hydratase gene mutations]] | ||
| | | | ||
* '''[[Multiple endocrine neoplasia type 2A]]''' | * '''[[Multiple endocrine neoplasia type 2A]]''' | ||
Revision as of 16:52, 31 July 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2] Mohammed Abdelwahed M.D[3]
Overview
Pheochromocytoma develops in called chromaffin cells, found in adrenal medulla which secrete adrenaline, noradrenaline. Familial pheochromocytoma may be caused by a mutation of either VHL, RET, NF1, SDHB or SDHD genes.
Causes
- Pheochromocytoma develops in called chromaffin cells, found in the adrenal medulla.
- Chromaffin cells typically secrete adrenaline, noradrenaline, and dopamine.
- These hormones are sympathetic stimulants.
- Pheochromocytoma results in the irregular and excessive release of these hormones causing hypertension and tachycardia. Approximately 10% are located in chromaffin tissue outside of the adrenal gland, The most common extra-adrenal locations are the abdomen and thorax
- Approximately 10% of tumors are malignant. Commonest sites of spread are the base of skull, head, and neck.
- The genetic base of pheochromocytoma depends on 2 clusters: cluster 1 tumors are noradrenergic. Cluster 2 tumors are adrenergic.[1]
| Cluster 1 | Cluster 2 |
|---|---|
|
References
- ↑ King KS, Pacak K (2014). "Familial pheochromocytomas and paragangliomas". Mol Cell Endocrinol. 386 (1–2): 92–100. doi:10.1016/j.mce.2013.07.032. PMC 3917973. PMID 23933153.