Phaeochromocytoma

Question 1

Define phaeochromocytoma

Answer 1

Tumour of the adrenal medulla that secretes catecholamines (NA/A), arising from chromaffin cells.

May also be extra-adrenal, found by the aortic bifurcation

Question 2

Aetiology of phaeochromocytoma

Answer 2

Sporadic

40% hereditary in adults e.g. MEN-2A/2B, neurofibromatosis, Von Hippel Lindau syndrome

Question 3

What is the 10% triad for phaeochromocytoma

Answer 3

10% extra-adrenal
10% malignant
10% bilateral

Question 4

Classic triad of phaeochromocytoma

Answer 4

Headache
Sweating
Tachycardia

Question 5

Presenting symptoms of phaeochromocytoma

Answer 5

Headache
Diaphoresis 
Palpitations 
Panic attack/sense of doom/anxiety/hyperactivity/confusion
Diarrhoea
Fever
Syncope
Angina
Dyspnoea

Question 6

Signs of phaeochromocytoma

Answer 6

Hypertension (paroxysmal)
Tachycardia
Pallor
Retinopathy 
Orthostatic hypotension
Hypercalcaemia

Question 7

Investigations of phaeochromocytoma

Answer 7

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

Question 8

Treatment for phaeochromocytoma

Answer 8

1. Alpha blocker with IV fluid e.g. phenoxybenzamine
2. Beta blocker e.g. atenolol
3. laparascopic adrenalectomy