Phaeochromocytoma

Question 1

what is a phaeochromocytoma?

Answer 1

a tumour of the chromatin cells - leading to the secretion of unregulated and excessive amounts of adrenaline.

Question 2

what is adrenaline?

Answer 2

a catecholamine hormone that stimulates the sympathetic nervous system and is responsible for fight or flight responses.
Increases HR, muscle tone, arousal, BP.

Question 3

what is the 10% rule for phaeochromocytoma?

Answer 3

10% is familial (MEN2)
10% is cancerous
10% are bilateral
10% occur outside the adrenal gland

Question 4

what age is phaeochromocytoma most common?

Answer 4

3rd and 5th decades

Question 5

what are the signs of phaeochromocytoma?

Answer 5

Fluctuating signs:

anxiety
sweating
panic attacks 
headaches
tremor
hypertension
arrhythmias
palpitations
tachycardia 
paroxysmal AF
weight loss
flushing
pyrexia

Question 6

how is phaeochromocytoma diagnosed?

Answer 6

Using 24hr urinary monitoring of catecholamines/metanephrines.

OR

Plasma free metanephrines

Question 7

why are metanephrines measured?

Answer 7

these are the breakdown product of adrenaline and are present in the blood for much longer

Question 8

what is the management for phaeochromocytoma?

Answer 8

FIRST: medical management:

alpha blocker - phenoxybenzamine

Then introduce a beta blocker (pronanolol)

THEN

surgery - removal of the adrenal gland is the definitive management.