What is a pheochromocytoma? What are the symptoms of this disorder? What is the pathophysiological basis...

What is a pheochromocytoma? What are the symptoms of this disorder? What is the pathophysiological basis for these symptoms?

Expert Solution

Pheochromocytoma is a neuroendocrine tumor of the medulla of adrenal glands or extra adrenal chromaffin tissue that failed to involute after birth.That secretes a high amount of catecholamines ,mostly norepinephrine with some epinephrine as well.

Pheochromocytoma also known as paragangliomas (extra adrenal) - which is tumor originates in ganglia of the sympathetic nervous system.

symptoms -

sympathetic nervous system hyperactivity including skin sensation, flank pain , elevated heart rate , elevated blood pressure - orthostatic hypertension ( a fall in systolic blood pressure greater than 20 mmHg or a fall in diastolic blood pressure greater than 10 mmHg upon standing).

palpitations , anxiety , panic attack, pallor , weight loss , excessive sweating (diaphoresis) , headache.

localized amyloid deposition , elevated blood glucose level .

(symptoms are in bold are most common)

pathophysiology -

Mutations in the gene VHL , RET ,NF1 ,SDHB .SDHD are well known to cause pheochromocytoma.

The disease is mainly a tumor of the multiple endocrine neoplasia syndrome - type IIA(MEN IIA) and type IIB(MEN IIB). Pheochromocytoma linked to MEN II can be acused by RET oncogene mutations.

gladiator answered 2 years ago

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