Phaeochromocytoma

Question 1

What is a phaeochromocytoma?

Answer 1

Adrenal medullary tumour which secretes catecholamines

Question 2

Name 2 catecholamines

Answer 2

Adrenaline
Noradrenaline

Question 3

What gene is associated with phaeochromocytoma?

Answer 3

MEN 2a and 2b

Question 4

Other than MEN 2a +2b, what else is associated with phaeochromocytoma?

Answer 4

neurofibromatosis I (tumours deposited along nerve myelin sheath)

Question 5

What are symptoms of phaechromocytoma?

Answer 5

Hypertension
Pallor
Very sweaty
Tachycardia

Question 6

How is pheochromocytoma diagnosed?

Answer 6

Raised plasma metanephrines and normetanephrines

(also could measure urinary catecholamines and CT to image tumour)

Question 7

How is phaeochromocytoma treated?

Answer 7

First give alpha blocker (eg phenoxybenzamine)

Then beta-blocker (eg atenolol)

If surgery is possible, excise tumour

Question 8

What is a complication of phaeochromocytoma?

Answer 8

Hypertension crisis (over 120/80)

Question 8

What can cause a hypertension crisis with phaeochromocytoma?

Answer 8

XR contrast
Opiates
TCA

Question 9

How is a hypertension crisis treated in phaeochromocytoma?

Answer 9

Phentolamine (alpha blocker)