Phaeochromocytoma

Question 1

Define phaeochromocytoma

Answer 1

Catecholamine-producing tumour that usually arise from chromaffin cells of the adrenal medulla but are extra renal in about 10% cases

Question 2

Distribution of phaeochromocytomas

3

Answer 2

10% are bilateral
10% are malignant
Extra adrenal phaeochromocytoma = paragangliomas

Question 3

Aetiology of phaeochromocytoma

3

Answer 3

Sporadic cases of unknown aetiology 
Familial in up to 30%
Familial seen in patients with:
MEN-2a
von Hippel-Lindau syndrome
Neurofibromatosis type 1

Question 4

Epidemiology of phaeochromocytomas

2

Answer 4

RARE

<0.2% of hypertensive patients

Question 5

Presenting symptoms of phaeochromocytoma

4 groups

Answer 5

General
Cardiorespiratory
GI
Neuropsychiatric

Question 6

Presenting symptoms of phaeochromocytoma - general

3

Answer 6

PAROXYSMAL episodes
Headache (due to malignant hypertension)
Sweating

Question 7

Presenting symptoms of phaeochromocytoma - cardiorespiratory
(3)

Answer 7

Palpitations
Chest pain
Dyspnoea

Question 8

Presenting symptoms of phaeochromocytoma - GI

3

Answer 8

Epigastric pain
Nausea
Constipation

Question 9

Presenting symptoms of phaeochromocytoma - neuropsychiatric

3

Answer 9

Weakness
Tremor
Anxiety

Question 10

Signs of phaeochromocytoma on physical examination

6

Answer 10

Hypertension
Postural hypotension
Pallor
Tachycardia
Fever
Weight loss

Question 11

Investigations for phaeochromocytoma

6

Answer 11

24hr urine collection - check catecholamine levels (& fractionated metanephrine levels)
Plasma free metanephrines
Tumour localisation (MRI or CT)
I-MIBG scintigraphy (other visualisation)
Screen for associated conditions
Genetic testing