2: Phaeochromocytoma

Question 1

What is a pheochromocytoma

Answer 1

Tumour of adrenal medulla

Question 2

What % of phaeochromocytomas are benign

Answer 2

90

Question 3

What % of phaeochromocytomas are malignant

Answer 3

10

Question 4

What % of phaeochromocytomas are familial

Answer 4

10

Question 5

What 3 genetic syndromes are associated with pheochromocytoma

Answer 5

• MEN IIa
• Von Hippel Lindau
• Neurofibromatosis

Question 6

Describe symptoms of phaechromocytoma

Answer 6

Episodic:

• HTN (90%)
• Sweating
• Palpitations
• Tachycardia
• Angor amine = sensation about to die

Question 7

What is the main symptom of phaechromocytoma

Answer 7

HTN (90%)

Question 8

Where do phaechromocytomas arise

Answer 8

Chromaffin cells

Question 9

Explain rule of 10% in pheochromocytoma

Answer 9

• 10% Malignant
• 10% Bilateral
• 10% Extra-adrenal

Question 10

If outside of the adrenal medulla, what is the most common site of phaeochromocytomas

Answer 10

Organ of zuckerkandl = adjacent to aortic bifurcation

Question 11

What is first-line investigation for pheochromocytoma

Answer 11

24h Urinary Metanephrines

Question 12

What is second-line investigation of pheochromocytoma

Answer 12

CT Abdomen

Question 13

What is mnemonic to remember management of pheochromocytoma

Answer 13

ABC

Question 14

Outline management of pheochromocytoma

Answer 14

Alpha -blocker

Beta-blocker

Cut it out

Question 15

Why is an alpha-blocker given first

Answer 15

To block peripheral effect of catecholamines

Question 16

What is a B-Blocker CI to give before a-blocker

Answer 16

B-blockers if given first will cancel out vasodilatory effects of B2 receptors, which will cause unopposed a-adrenergic stimulation of vasoconstriction