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)
Abundant chromaffin cells
Tumour of the adrenal medulla.