Pheochromocytoma causes: Difference between revisions
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==Overview== | ==Overview== | ||
Pheochromocytoma develops | Pheochromocytoma develops from [[chromaffin cells]], found in [[adrenal medulla]] which secrete [[adrenaline]], [[noradrenaline]], and [[Dopamine|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 [[Norepinephrine|noradrenergic]] and cluster 2 tumors are [[adrenergic]]. Familial pheochromocytoma may be caused by a mutation of either ''[[SDHD]],'' ''[[VHL]]'', ''[[SDHB]],'' ''[[RET proto-oncogene|RET]]'', ''[[NF1]]'' genes. | ||
==Causes== | ==Causes== | ||
* Pheochromocytoma develops in called [[chromaffin cells]], found in the [[adrenal medulla]]. | * Pheochromocytoma develops in called [[chromaffin cells]], found in the [[adrenal medulla]]. | ||
Revision as of 17:34, 15 August 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 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 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 |
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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.