Pheochromocytoma causes
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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 | |
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| 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.