Phaeochromocytoma is a rare catecholamine secreting tumour. About 10% are familial and may be associated with MEN type II, neurofibromatosis and von Hippel-Lindau syndrome

Basics (rule of 10’s)

• bilateral in 10%
• malignant in 10%
• extra-adrenal in 10% (most common site = organ of Zuckerkandl, adjacent to the bifurcation of the aorta)

Features are typically episodic

• hypertension (around 90% of cases, may be sustained)
• headaches
• palpitations
• sweating
• anxiety

Tests

• 24 hr urinary collection of metanephrines (sensitivity 97%*)
• this has replaced a 24 hr urinary collection of catecholamines (sensitivity 86%)

Surgery is the definitive management. The patient must first however be stabilized with medical

management:

• alpha-blocker (e.g. phenoxybenzamine), given before a
• beta-blocker (e.g. propranolol)