Phaeochromocytoma

Question 1

What is a Phaeochromocytoma?

Answer 1

Catecholamine-producing tumours that usually arise from chromaffin cells of the adrenal medulla

Question 2

Describe the aetiology of Phaeochromocytoma

Answer 2

adrenal paraganglioma or a chromaffin cell tumor
NOT ADENOMA
~10% extra-adrenal
10% bilateral
10% malignant
Sporadic cases are of unknown aetiology
Familial in up to 30%

Question 3

In what 4 conditions may familial cases of Phaeochromocytoma be seen?

Answer 3

MEN2a + MEN2b
von Hippel-Lindau syndrome
Neurofibromatosis type 1
STHB or STHD

Question 4

Describe the epidemiology of Phaeochromocytoma

Answer 4

RARE

<0.2% of cases of hypertensive patients

Question 5

Describe general symptoms of Phaeochromocytoma

Answer 5

PAROXYSMAL episodes: comes in attacking manner
Headache (due to malignant HTN)
Sweating

Question 6

List 3 cardiorespiratory symptoms of Phaeochromocytoma

Answer 6

Palpitations
Chest pain
Dyspnoea

Question 7

List 3 GI symptoms of Phaeochromocytoma

Answer 7

Epigastric pain
Nausea
Constipation

Question 8

List 3 neuropsychiatric symptoms of Phaeochromocytoma

Answer 8

Weakness
Tremor
Anxiety

Question 9

List 6 signs of Phaeochromocytoma

Answer 9

HTN 
Postural hypotension  
Pallor 
Tachycardia  
Fever  
Weight loss

Question 10

How do Phaeochromocytoma attacks differ to anxiety attacks?

Answer 10

Phaeo: high BP but not tachycardia.
Anxiety: both high BP + tachycardia.

Question 11

What happens to adrenaline levels in Phaeochromocytoma? What is the consequence of this?

Answer 11

Adrenaline secretion isn’t constant

Completely asymptomatic between attacks.

Question 12

What investigations are performed in suspected Phaeochromocytoma?

Answer 12

24 hr urine collection: catecholamine levels (+ check for fractionated metanephrine)

Plasma free metanephrines + catecholamines

Genetic testing (if <50 mostly)

Question 13

What are metanephrines?

Answer 13

metabolites of adrenaline

Question 14

What other testing may be performed in confirmed Phaeochromocytoma?

Answer 14

Genetic tests for associated conditions  
Tumour localisation (MRI or CT adrenal protocol)

Question 15

What is the management of someone with suspected Phaeochromocytoma once the functional tests come back?

Answer 15

1. Alpha blockade
2. Send for imaging
3. B-blocker: if develops a reflex tacchycardia (not all will require a B-blockade)
4. Monitor BP + slowly increase A-blockade (few weeks)
5. Surgical excision of adrenal gland

Question 16

What are adrenal adenomas? Give 3 conditions in which these are found

Answer 16

tumours arising from the cortex
Cushing’s syndrome
Conn’s
Testosterone producing

Question 17

What are extra-adrenal phaeochromocytomas are referred to as?

Answer 17

paragangliomas