Phaeochromocytoma

Question 1

what is it

Answer 1

rare catecholamine producing tumour

Question 2

what does it arise from

Answer 2

arise from sympathetic paraganglia cells - collection of chromaffin cells. usually found within the medulla. extra renal tumours rarer (by aortic bifurcation)

Question 3

what are 90% due to

Answer 3

sporadic

Question 4

what are 10% due to

Answer 4

part of hereditary cancer syndromes, eg thyroid, MEN-2a and 2b, neurofibromatosis, von Hippel Lindau

Question 5

what is the classic triad

Answer 5

episodic headache, sweating, tachycardia

Question 6

tests

Answer 6

WCC, plasma and urines for free metadrenaline and normetadrenaline. localisation- CT/MRI. of MIBG chromaffin seeking isotrope scan

Question 7

treatment

Answer 7

a blockade pre op, b blockers if heart disease or tachycardic, post op- urine metadrenalines.

Question 8

emergency what do you do

Answer 8

combined a and b blockade, long acting a blocker eg phenoxybenzamine. b blocker. surgery for full alpha blockade and volume expansion

Question 9

what happens in a phaeochromocytoma crisis

Answer 9

acute hypertensive crises- exacerbated symptoms by stress, abdo palpation, parturition, general anaesthetic, contrast media used in imaging

Question 10

signs in crisis

Answer 10

pallor, pulsating headache, hypertension, feels ‘about to die’, pyrexial. ECG- signs LVF, incr ST segment, VT and cardiogenic shock