Background/Epidemiology
- Rare neuro-endocrine tumours that secrete catecholamines (adrenaline and noradrenaline).
- Around 80% occur in the adrenal medulla (Phaeochromocytoma) and 20% occur elsewhere in the body in sympathetic ganglia (paragangliomas).
- Majority are benign but up to 15% show malignant features
- Around 30% are associated with inherited disorders e.g. neurofibromatosis, von Hippel-Lindau; MEN2
Clinical Features
- Hypertension (NB <0.1% of hypertensives)
- Often marked postural hypotension
- May be paroxysmal
- Episodes of pallor/flushing; palpitations; sweating; headache; anxiety/butterflies
- Weight loss
- Usually presents as an acute episode relating to excess catecholamine/hypertension e.g. stroke, MI, left ventricular failure, hypertensive retinopathy or accelerated phase hypertension
Investigations
- Urinary catecholamine metabolites (namely metanephrine) can be measured
- High false positive so repeat is often required
- Imaging
- Abdominal CT
Management
- Although definitive management is surgical, medical management, particularly if the patient presents in the acute setting, is often required to stabilise the patient beforehand
- non-competitive α-blockade with phenoxybenzamine can be used (doxazosin can be used but due to competitive nature it will often not have a great effect)
- if this produces a tachycardia then propanolol can be added
- non-competitive α-blockade with phenoxybenzamine can be used (doxazosin can be used but due to competitive nature it will often not have a great effect)