Phaeochromocytoma Flashcards
Define phaeochromocytoma
Tumour of the adrenal medulla that secretes catecholamines (NA/A), arising from chromaffin cells.
May also be extra-adrenal, found by the aortic bifurcation
Aetiology of phaeochromocytoma
Sporadic
40% hereditary in adults e.g. MEN-2A/2B, neurofibromatosis, Von Hippel Lindau syndrome
What is the 10% triad for phaeochromocytoma
10% extra-adrenal
10% malignant
10% bilateral
Classic triad of phaeochromocytoma
Headache
Sweating
Tachycardia
Presenting symptoms of phaeochromocytoma
Headache Diaphoresis Palpitations Panic attack/sense of doom/anxiety/hyperactivity/confusion Diarrhoea Fever Syncope Angina Dyspnoea
Signs of phaeochromocytoma
Hypertension (paroxysmal) Tachycardia Pallor Retinopathy Orthostatic hypotension Hypercalcaemia
Investigations of phaeochromocytoma
24h urine for metanephrines and metadrenaline: elevated
Serum free metanephrines and normetenephrines: elevated
Abdominal CT/MRI for tumour imaging: tumour
MIBG: high uptake intensity
Plasma catecholamine: elevated
Genetic testing for VHL syndrome, MEN1 and MEN2
Treatment for phaeochromocytoma
- Alpha blocker with IV fluid e.g. phenoxybenzamine
- Beta blocker e.g. atenolol
- laparascopic adrenalectomy