Phaeochromocytoma

Question 1

What are catecholamines

Answer 1

Epinephrine and norepinephrine

Question 2

What genetic conditions are associated with Phaeochromocytoma? (3)

Answer 2

Men 2A
Men 2B
VHL - Von Hippel Lindau

Question 3

Pheaochromocytoma is a catecholamine-producing tumour of the neural crest arising from _______ Cells of the adrenal _______(medulla or cortex)

Answer 3

Chromaffin cells arising from the medulla

Everything else wrong with the adrenal gland is the cortex (Zona glomerulosa, Fasciculata, and reticularis)

Question 4

What is the characteristic triad of Phaeochromocytoma?
How would a patient present?

Answer 4

Question 5

What are your 3 main ddx

Answer 5

Catecholamine-secreting Paragangliomas (same but arise from different cells)
Uncontrolled HTN
Thyrotoxicosis/severe hyperthyroidism
Conn’s Syndrome

Question 6

What test is best used to locate metastatic or ectopic disease for phaeochromocytoma?

Answer 6

MIBG Radiolabelled iodine uptake scan

Question 7

A patient presents with episodic headaches, sweating, tachycardia, tremor, and hypertension. What is the first-line diagnostic test you need to perform?

What else?

Answer 7

Question 8

A patient with known phaeochromocytoma needs hip replacement. What is the pre-op regimen the patient must go through ahead of their planned surgery?

What happens if they dont?

Answer 8

Alpha-adrenergic blockade 10-14 days before to control HTN
Beta blockage (beta blockers) 2-3 days pre-op

If they dont, Vtach -> Vfib -> death

Question 9

What weird drugs should patients with phaeochromocytoma not take

Answer 9

Metoclopramide
Glucagon
Histamine
yikes

Question 10

What is the absolute management of a patient with phaeochromocytoma if it was:
Unilateral:
Bilateral:

Answer 10