Phaeochromocytoma

Question 1

What is a phaechromocytoma?

Answer 1

A tumour arising from catecholamine-producing chromaffin cells of the adrenal medulla that classically presents with:

• headaches
• diaphoresis
• palpitations

in the setting of paroxysmal hypertension.

Symptoms are usually episodic and tend to progress as the tumour grows.

The majority of these tumours are benign.

About 80% to 90% arise in the adrenal gland; the remainder being extra-adrenal in origin and most commonly found in the head and neck.

These tumours mostly develop sporadically.

Up to 40% of cases are a manifestation of a hereditary syndrome, such as multiple endocrine neoplasia type 2 or Von Hippel-Lindau syndrome.