Pheochromocytoma causes: Difference between revisions
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==Causes== | ==Causes== | ||
* Pheochromocytoma arises from [[chromaffin cells]], found in the [[adrenal medulla]]. | * Pheochromocytoma arises from [[chromaffin cells]], found in the [[adrenal medulla]]. | ||
* Approximately 10 | * Approximately 10 percent are located in [[Chromaffin cell|chromaffin tissue]] outside of the [[adrenal gland]]. The most common extra-[[Adrenal gland|adrenal]] locations are the [[abdomen]] and [[thorax]]. | ||
* [[Chromaffin cells]] typically secrete [[adrenaline]], [[Norepinephrine|noradrenaline]], and [[dopamine]]. These hormones are [[Sympathetic nervous system|sympathetic]] [[Stimulant|stimulants]]. | * [[Chromaffin cells]] typically secrete [[adrenaline]], [[Norepinephrine|noradrenaline]], and [[dopamine]]. These hormones are [[Sympathetic nervous system|sympathetic]] [[Stimulant|stimulants]]. | ||
* Pheochromocytoma results in the irregular and excessive release of these [[hormones]] causing [[hypertension]] and [[Tachycardia|tachycardia.]] | * Pheochromocytoma results in the irregular and excessive release of these [[hormones]] causing [[hypertension]] and [[Tachycardia|tachycardia.]] | ||
* Approximately 10 | * Approximately 10 percent of [[Tumor|tumors]] are [[malignant]]. Common sites of spread are the base of [[skull]], [[head]], and [[neck]]. | ||
* 50-60 percent of pheochromocytomas are sporadic, others are familial. | * 50-60 percent of pheochromocytomas are sporadic, others are familial. | ||
* Familial forms can be sub-divided into two major clusters based on [[genes]] causing the disease.<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> | * Familial forms can be sub-divided into two major clusters based on [[genes]] causing the disease.<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> | ||
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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 arises from chromaffin cells, found in adrenal medulla which secrete adrenaline, noradrenaline, and dopamine. 50-60 percent of pheochromocytomas are sporadic, others are familial. Familial forms can be sub-divided into two major clusters based on genes causing the disease. Cluster 1 tumors are noradrenergic and cluster 2 tumors are adrenergic. Familial pheochromocytoma may be caused by a mutation of either SDHD, VHL, SDHB, RET, NF1 genes.
Causes
- Pheochromocytoma arises from chromaffin cells, found in the adrenal medulla.
- Approximately 10 percent are located in chromaffin tissue outside of the adrenal gland. The most common extra-adrenal locations are the abdomen and thorax.
- 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 percent of tumors are malignant. Common sites of spread are the base of skull, head, and neck.
- 50-60 percent of pheochromocytomas are sporadic, others are familial.
- Familial forms can be sub-divided into two major clusters based on genes causing the disease.[1]
| Familial pheochromocytoma | |
|---|---|
| Cluster 1 (Noradrenergic) | Cluster 2 (Adrenergic) |
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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.