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Pheochromocytoma:

A catecholamine-producing tumour derived from the chromaffin cells (of the adrenal medulla/ outside the adrenal medulla)

  • Dusky coloured tumor
  • 10% tumour
    • bilateral disease (10%)
    • malignant (10%)
    • located in chromaffin tissue OUTSIDE the adrenal gland (10%)
    • arise in childhood (10%)
    • familial (10%)
    • recur after being resected (10%)
  • Can be inherited as an autosomal dominant trait & may be part of multiple endocrine neoplasia (MEN)
    • MEN 2A & 2B

Can manifest:

  • hypertension (but surgically curable – resection)
  • Cardiac manifestations
  • metabolic problems
  • neuropsychiatric problems – anxiety
  • renal artery stenosis
  • cholelithiasis (gallstones)

Clinical features: 5 ‘P’s

  • Pressure – hypertension
  • Pain – headache
  • Perspiration – diaphoresis (excessive sweating)
  • Pallor – vasoconstriction
  • Palpitations – tachyarrhythmias

Diagnosis:

  • Raised 24 hour urinary excretion of catecholamines (NE, E)
  • Raised catecholamine metabolites (metanephrine, normetanephrine, 4-hydroxy-3-methoxymandellic acid (HMMA)/(VMA)

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Abundant chromaffin cells

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Tumour of the adrenal medulla.

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