Phaeochromocytoma

• 10% are part of a hereditary cancer syndrome e.g. MEN2a, MEN2b, neurofibromatosis, Von Hippel-Lindau syndrome

Symptoms

• Hypertension, anxiety, chest tightness

Tests

• Screening
  • 3 x 24hr urine collections for free catecholamine
• Localisation
  • Abdo CT/MRI of a MIBG chromaffin-seeking isotope scan (useful for detecting extra-adrenal tumours)

Treatment

• Surgery
• ? and ? blockade pre-op. The ? blocker is given before the ? to avoid a crisis from unopposed ?-adrenergic stimulation
• Post op – do 24hr catecholamines 2wk post op and monitor BP
• Lifelong follow up as malignant recurrence may present late

Complications

• Heart failure, dilated cardiomyopathy, arrhythmias, stroke and death due to hypertensive crisis

Management of phaeochromocytoma emergencies

• Stress, abdominal palpation, parturition, general anaesthetic or contrast media used in radiography may produce danger hypertensive crisis

Treatment

• Phentolamine 2-5mg IV – repeat to maintain safe BP
• Labetolol is an alternative
• When BP is controlled give phenoxybenzamine 10mg/24hr PO (increase by 10mg/day as needed max is 0.5-1mg/kg/12hr
• SE – postural hypotension, dizziness, tachycardia, nasal congestion, miosis
• A ?-blocker can be given at this stage to control any tachycardia

• Surgery is generally done electively after a period of 4-6weeks to allow full ? blockage and volume expansion