Phaeochromocytoma

Question 1

Where is adrenaline produced in the body?

Answer 1

Chromaffin cells, in the adrenal glands

Question 2

What is a phaeochromocytoma?

Answer 2

A tumour of the chromaffin cells which produces excessive amounts of adrenaline in bursts

Question 3

Phaeochromocytoma is associated with which rare inherited condition?

Answer 3

Multiple endocrine neoplasia type 2 (MEN2)

Question 4

Describe the clinical features of phaeochromocytoma

Answer 4

Signs and symptoms tend to fluctuate with peaks and troughs related to when the tumour is secreting adrenaline (the tumour releases adrenaline in bursts):

• Sweating
• Anxiety
• Tachycardia and palpitations
• Hypertension

Question 5

Describe the investigation of phaeochromocytoma

Answer 5

24 hour urine metanephrines

Question 6

Describe the management of phaeochromocytoma

Answer 6

Patients should have symptoms controlled medically with alpha and/or beta blockers, before definitive surgical management (adrenalectomy)

Question 7

What is meant by the 10% rule (with reference to phaeochromocytoma)?

Answer 7

There is a 10% rule to describe the patterns of tumour:

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