Phaeochromocytoma

Question 1

Define:

Answer 1

Catecholamine producing tumour usually arising from chromaffin cells of the adrenal gland but can be extra-adrenal.

Question 2

Aetiology/risk factors:

Answer 2

10% bilateral
10% malignant
10% familial - MEN 2a + b, Hippel-lindau syndrome + neurofibromatous type 1

Dangerous as can cause severe hypertension

Question 3

Epidemiology:

Answer 3

rare

<0.2 % of hypertensive patients

Question 4

Symptoms:

Answer 4

Paroxysmal episodes

CLASSIC TRIAD:

1. episodic headaches
2. sweating
3. tachycardia

Cardiorespiratory:

• Palpitations
• Chest pain
• Dyspnoea
• Faints

CNS:

• Visual disturbances
• Headaches
• Dizziness
• Tremor

GI:

• epigastric pain
• nausea
• constipation/ D + V

Neuro:

• Depression
• Tremor
• anxiety
• confusion
• panic

Question 5

Signs:

Answer 5

Hypertension 
Postural hypotension 
Pallor 
Tachycardia 
Fever
Weight loss

Question 6

Investigations:

Answer 6

24hr urine collection - check for catecholamines and metenpherines

Tumour localisation - CT or MRI

Screen for associated symptoms

Genetic testing

Plasma free metenephrines

i-MIBG scintigraphy (seeing tumours)