Phaeochromocytoma/Paraganglioma

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

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