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rare catecholamine secreting tumour.
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 sustatined)
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)