15.11 Adrenal Medulla

Question 1

what kind of cells compose the adrenal medulla?

Answer 1

neural crest-derived chromaffin cells

Question 2

main physiologic source of catecholamines (epinephrine and norepinephrine)

Answer 2

adrenal medulla

Question 3

how do you diagnose pheo?

Answer 3

increased serum metanephrines and increased 24 hours urine metanephrines and vanillylmandelic acid (VMA)

Question 4

how do you treat pheo?

Answer 4

surgical excision w/ phenoxybenzamine administered perioperatively

Question 5

what is phenoxybenzamine?

Answer 5

irreversible alpha-blocker (prevents hypertensive crisis)

Question 6

why administer phenoybenzamine perioperatively with removal of a pheochromocytoma?

Answer 6

it prevents hypertensive crisis in the event that manipulation of the tumor releases catecholamines into the bloodstream

Question 7

what is the rule of 10 with regard to pheochromocytoma?

Answer 7

10% bilateral
10% familial
10% malignant
10% located outside the adrenal medulla

Question 8

the 10% of pheos located outside the adrenal medulla are often located in the

Answer 8

bladder wall or organ of Zuckerkandl at the IMA root

Question 9

pheos are associated w/ what four genetic conditions

Answer 9

1. MEN2A
2. MEN2B
3. von Hippel-lindau disease
4. Neurofibromatosis type 1

Question 10

with what is von hippel lindau associated?

Answer 10

pheochromocytoma, RCC, hemangioblastoma of the cerebellum