Endocrinology - Phaeochromocytoma

Question 1

What is a phaeochromocytoma?

Answer 1

A tumour of the chromaffin cells in the adrenal medulla of the adrenal glands

These cells secrete adrenaline, the tumour causes excess secretion

Question 2

10% rule of phaeochromocytoma

Answer 2

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

Question 3

Phaeochromocytoma is associated with

Answer 3

Multiple endocrine neoplasia type 2 (MEN 2)

25% phaeos are familial and associated with MEN2

Question 4

Presentation of phaeochromocytoma

Answer 4

Anxiety
Sweating
Headache
Hypertension
Palpitations, tachycardia and paroxysmal atrial fibrillation

Question 5

Diagnosis of phaeochromocytoma

Answer 5

24 hour urine catecholamines

Plasma free metanephrines (a breakdown product of adrenaline)

These would both be expected to be high

Question 6

Management of phaeochromocytoma

Answer 6

Alpha blockers (i.e. phenoxybenzamine)

Beta blockers once established on alpha blockers

Adrenalectomy to remove tumour is the definitive management

Symptoms should be controlled medically before surgery to reduce risk of the anaesthetic/surgery