Phaeochromocytoma

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

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)

+PassMedicine Notes

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