Phaeochromocytoma

• Tumour of sympathetic nervous system
• 1:1000 cases of hypertension
• 90% in adrenal medulla, <10% elsewhere
• 25% multiple
• 10% malignant
• Most Associated With Multiple Endocrine Neoplasia (MEN) sydromes
• Secrete both adrenaline and noradrenaline but large ones  secrete almost all noradrenaline

Pathology

• brown solid nodules

History

• related to catecholamine excess
• consider in cardiovascular instability and severe hypertension in pregnancy
• General
  • Weight loss
  • Sweating
  • Flushing
• Hands
  • Tremor
  • Raynaud's Phenomenon
• CNS
  • Anxiety / Panic attacks
  • Headache
• CVS
  • Chest Pain
  • Palpitations
• GI
  • Nausea +/- vomiting
  • Constipation / Diarrhoea
• Renal
  • Polyuria / Nocturia

Examination

• General
  • Fever
  • Pallor / Flushing
• CVS
  • Tachycardia + arrythmias
  • Bradycardia
  • Hypertension
    • intermittent
    • constant
  • Postural hypotension
• GU
  • Glycosuria

Investigations

• Urinary Metabolites
  • metanephrines (preferred)
  • vanillylmandelic acid 
• Plasma / Urinary catecholamines
• CT scans / MRI
• MIBG isotope scans 
  • sympathetic activity
  • 90% success rate

Treatment

• Anxiety Management
• Antidepressants
• Surgery
  • under complete alpha and beta blockade 
    1. phenoxybenzamine 20-80mg / day 
    2. propranolol 120-240 mg / day
    3. transfusion of whole blood to expand plasma volume

Prognosis

• Non-malignant tumours 
  • 95% 5 year survival
• 10% recurrence / further tumours

Complications

• Dangers of anaesthetics
  • hypertension and hypotension
  • sodium nitroprusside must be available to counter sudden severe hypertension

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