Phaeochromocytoma

Question 1

What is a Phaeochromocytoma?

Answer 1

A neuroendocrine tumour of the adrenal medulla, specifically the chromaffin cells

That secretes catecholamines

Question 2

What are the causes of Phaeochromocytoma?

Answer 2

The 10% rule!

10% malignant
10% bilateral
10% familial
10% extra-adrenal

Question 3

Name a familial disease that causes Phaeochromocytoma?

Answer 3

Von Hippel Lindau disease

Question 4

What are the catecholamines secreted by adrenal medulla?

Answer 4

Noradrenaline (mainly)
Adrenaline
(dopamine - only a small amount is released by adrenal medulla)

Question 5

Clinical features of Phaeochromocytoma?

Answer 5

Hypertension
Sweating
Hyperglycaemia

Symptomatic episodes:

• Pallor
• Palpitations
• Panic
• Pain in chest

Question 6

Investigation of Phaeochromocytoma?

Answer 6

Urinary catecholamines: see high levels

Raised catecholamines in plasma

Question 7

Management of Phaeochromocytoma?

Answer 7

Medical: control BP

• alpha blockers
• calcium channel blockers

Surgical resection

Question 8

What special considerations do patients undergoing surgery to resect a Phaeochromocytoma need?

Answer 8

They need to be given alpha blockers before, to stagger loss of adrenaline

Sudden loss of adrenaline by removing the tumour in surgery can cause a stroke