Phaeochromocytoma in afya

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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)

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