Phaeochromocytoma

Question 1

define

Answer 1

this is a tumour of the adrenal medulla that produces excess catecholamines

Question 2

presentation

Answer 2

insidious onset
triad of hypertension, headache and sweating
postural hypotension
palpitations
metabolic syndromes
pallor, tachycardia, anxiety
weight loss

Question 3

what is this tumour called if it is on the sympathetic chain (extra-adrenal)

Answer 3

paraganglioma (aortic bifurcation)

Question 4

complications of a paraganglioma

Answer 4

LVF
myocardial necrosis
stroke
shock
paralytic ileus of bowel

Question 5

diagnosis

Answer 5

confirm catecholamine excess
hyperglycaemia, potentially low K+, haemocrit, lactic acidosis
CT, MRI, MIBG (radioactive iodine) and PET
AMBP/HMBP as can have paroxysmal hypertension

Question 6

how do you confirm catecholamine excess

Answer 6

urine 2x24 hour catecholamines or metanephrines (confounding substances) or plasma (ideally at the time of symptoms)
catecholamines in urine

Question 7

who should be investigated for phaeochromocytoma

Answer 7

family members
resistant hypertension
<50 with hypertension
classic presentation

Question 8

management

Answer 8

alpha blockers before beta blockers
fluid and/or blood replacement
surgery and chemo with MIBG if malignant

Question 9

pitfalls

Answer 9

catecholamines are raised in CHF, may be episodic secretion