Pheochromocytoma

Question 1

What is a pheochromocytoma?

Answer 1

Tumour of the chromaffin cells that secrete unregulated and excessive amounts of adrenaline

Question 2

What are these tumour associated with?

Answer 2

MEN 2- Multiple Endocrine Neoplasia type 2

Question 3

What is the 10% rule of this tumour?

Answer 3

10% bilateral
10% cancerous
10% outside the adrenal gland

Question 4

What is the presentation of pheochromocytoma?

Answer 4

Signs and symptoms fluctuate relating to the bursts of adrenaline secretion:
- Anxiety
- Sweating
- Headache
- Hypertension
- Palpitations, tachycardia and paroxysmal atrial fibrillation

Question 5

How is pheochromocytoma diagnosed?

Answer 5

24 hr urine catecholamines
Plasma free metanephrines (breakdown product of adrenaline)

Question 6

What is the management of pheochromocytoma?

Answer 6

Alpha blockers
Beta blockers
Adrenalectomy to remove the tumour

Question 7

What 3 conditions are associated with MEN II?

Answer 7

Pheochromocytoma
Primary hyperparathyroidism
Medullary thyroid cancer