Phaeochromocytoma

Question 1

What is a phaeochromocytoma?

Answer 1

A rare catecholamine secreting tumour.

Question 2

How many of these are familial?

Answer 2

10%

Question 3

What conditions is this associated with?

Answer 3

• MEN type 2
• neurofibromatosis
• von Hippel-Lindau syndrome

Question 4

How many of these are bilateral?

Answer 4

10%

Question 5

How many of these are malignant?

Answer 5

10%

Question 6

How many of these are extra-adrenal?

Answer 6

10%

Question 7

What is the most common extra-adrenal site?

Answer 7

Organ of Zuckerkandl, adjacent to the bifurcation of the aorta

Question 8

How regular are the symptoms?

Answer 8

Episodic

Question 9

Symptoms?

Answer 9

• hypertension (around 90% of cases, may be sustained)
• headaches
• palpitations
• sweating
• anxiety

Question 10

Main diagnostic test?

Answer 10

24 hr urinary collection of metanephrines (sensitivity 97%*)

Question 11

What other test can be performed?

Answer 11

24 hr urinary collection of catecholamines (sensitivity 86%)

Question 12

Definitive management?

Answer 12

Surgery

Question 13

What must be done before patient is given definitive management?

Answer 13

Patient must be stabilised with medical management:
• alpha-blocker (e.g. phenoxybenzamine), given before a
• beta-blocker (e.g. propranolol)