Phaeochromocytomas are rare tumours of chromaffin cells which produce catecholamines.
Presenting features may include:
- hypertension
- headache
- palpitations
- diaphoresis (=excessive sweating)
- nausea and vomiting
10% are familial, as part of MEN II, neurofibrosis or Von Hippel Lindau syndrome
10% are bilateral, 10% are malignant and 10% are extraadrenal
Extraadrenal sites include the organ of Zuckerkandl and bladder.
Diagnosis is by 24 hour urinary VMA. Localisation is then by CT/MRI.
Management is surgical with pre operative alpha blockade (prazosin or phenoxybenzamine)
Small print gem: Metoclopramide must be avoided in patients with phaechromocytoma as it increases noradrenaline release via presynaptic dopamine receptor blockade, which in the presence of a phaechromocytoma can cause severe hypercatecholaminaemia.
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