Phaeochromocytoma

Question 1

Define Phaeochromocytoma

Answer 1

Catecholamine producing tumour of the adrenal medulla

Question 2

The majority of cases of Phaeochromocytoma are:

a) Benign
b) Malignant

Answer 2

a) Benign (80-85%)

Question 3

Give examples of catecholamine hormones

Answer 3

Adrenaline

Noradrenaline

Dopamine

Question 4

Define paraganglioma

Answer 4

Catecholamine producing tumours in sympathetic nerve tissue elsewhere in the body

Question 5

Describe the onset and timing of the clinical features of Phaeochromocytoma

Answer 5

Features tend to episodic as the hormone secretion tends to be in burst giving periods of worse symptoms followed by more settled periods

Question 6

Name some of the clinical features of Phaeochromocytoma

Answer 6

Catecholamines have an important role in the sympathetic nervous system as part of the ‘fight-or-flight’ response. Thus features mimic the signs of the “fight-or-flight” response

• Key feature: Episodic or sustained hypertension
• Headaches
• Palpitations
• Sweating
• Anxiety

Question 7

What is the key clinical feature of Phaeochromocytoma

Answer 7

Episodic or sustained hypertension

Question 8

What is the gold standard test to diagnose phaeochromocytoma

Answer 8

24 hr urinary collection of metanephrines

Question 9

What is the definitive management option for phaeochromocytoma

Answer 9

Surgical removal

Question 10

Name a potential complication of the surgical removal of phaeochromocytomas

Answer 10

Sudden hypotensive emergency resulting in cardiac arrest

Question 11

Which drug should have been given, before beta-blockade, to avoid the sudden cardiac arrest, a potential surgical complication of the removal of phaeochromocytoma

Answer 11

Alpha blocker e.g. phenoxybenzamine

Remember: PHaeochromocytoma - give PHenoxybenzamine before beta-blockers