Phaeochromocytoma

Question 1

What do phaeochromocytomas secrete?

Answer 1

catecholamines

Question 2

What genetic conditions is phaeochromocytoma associated with?

Answer 2

MEN type II
Neurofibromatosis
von Hippel-Lindau syndrome

Question 3

What percentage of phaeochromocytomas occur bilaterally?

Answer 3

10%

Question 4

What percentage of phaeochromocytomas are malignant?

Answer 4

10%

Question 5

What percentage of phaeochromocytomas are extra-adrenal? And, if so, where do they most commonly occur?

Answer 5

10%

• bifurcation of aorta
  (organ of Zuckerkandl)

Question 6

Describe the typical features seen in phaeochromocytoma

Answer 6

hypertension
headaches
palpitations
sweating
anxiety

Question 7

What investigation is used to diagnose phaeochromocytoma?

Answer 7

24 hr urinary collection of metanephrines

Question 8

What medications are used to manage phaeochromocytoma prior to eventual tumour removal surgery?

Answer 8

alpha-blocker (e.g. phenoxybenzamine)
THEN
beta-blocker (e.g. propranolol)