Phaeochromocytoma (3)

Question 1

What is it?

How does it present?
→ Why does it present like this?

What is it associated with?

Answer 1

➊ Catecholoamine-secreting tumour of the Chromaffin cells in the adrenal medulla, leading to high adrenaline

➋ • Episodic hypertension
• Headache, sweating, tachycardia
• Tremor
• Anxiety
→ Adrenaline is secreted in bursts, therefore pts have periods of worsened symptoms, followed by more settled periods

➌ Multiple Endocrine Neoplasia Type 2 (MEN2)

Question 2

What are the investigations to do?

What’s the definitive management option?
→ What can be given before this is done?

Answer 2

➊ • Plasma metanephrines followed by urinary metanehprines
• Imaging

➋ Surgical resection
→ a-blocker e.g. phenoxybenzamine, followed by a b-blocker to prevent hypertensive crises