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]

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 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 extradrenal locations are the abdomen and thorax
• Approximately 10% of tumors are malignant. Commonest sites of spread are base of skull, head and neck.
• Genetic base of pheochromocytoma depends on 2 clusters: cluster 1 tumors are noradrenergic. Cluster 2 tumors are adrenergic.^[3]

Cluster 1	Cluster 2
Succinate dehydrogenase (SDH) subunit genes Von Hippel-Lindau (VHL) disease Fumarate hydratase gene mutations	Multiple endocrine neoplasia type 2A Multiple endocrine neoplasia type 2B Neurofibromatosis type 1 (NF1)