phaechromocytoma Flashcards
what is a phaechromocytoma
a tumour of the chromaffin cells in the adrenal glands that produce excessive amounts of adrenaline
what type of hormone is adrenaline
a catecholamine hormone
what does adrenaline do
its a neurotransmitter that stimulates the sympathetic nervous system and is responsible for flight or flight response
how is adrenaline secreted by a phaechromocytoma
in bursts giving periods of worse symptoms by more settled periods
which genetic variation is a phaechromocytoma associated with
MEN 2
diagnosis of phaechomocytoma
- 24 hour urine catecholamines
- plasma free metanephrines
which diagnostic test is more reliable and why
metanephrines as they have a longer half life which makes them less prone to dramatic fluctuations so more reliable
presentation of phaechromocytoma
- anxiety
- sweating
- headache
- hypertension
- palpitations, tachycardia and paroxysmal atrial fibrillation
management for phaechromocytomas
- alpha blockers
- beta blockers once established on alpha blockers
- adrenalectomy to remove tumour is definitive management