Pheo

Question 1

Characteristics

Answer 1

Tumor of adrenal medulla in ADULTS
(neuroblastoma in kids)

From chromaffin cells (NC derived)

Question 2

Produces

Answer 2

NE, dopamine causing HTN

Urinary VMA (breakdown of NE and EPI) and plasma catecholamine level increased

Question 3

Associated with

Answer 3

NF type 1

MEN type 2A and 2B

Question 4

Rule of 10

Answer 4

10% malignant
10% bilateral
10% extra adrenal
10% calcify
10% kids

Question 5

Treatment:

Answer 5

tumor resection after effective alpha and beta block
irreversible alpha block first with henoxybenzamine to avoid HTN crisis.

beta blocker then given to slow HR

Question 6

Episodic hyperadrenergic symptoms

Answer 6

5P’s

Pressure
Pain (HA)
Perspiration
Palpitation (tachy)
Pallor

Question 7

Catecholamine pathways

Answer 7

L-dopa => dopa

Dopa => either HVA or NE
NE => either normetanephrine or VMA, or EPI
EPI => metanephrine, VMA

Question 8

Neuroblastoma

Answer 8

1) Most common tumor of adrenal medulla in chilren
2) Occur anywhere in sympathetic chain.
3) HVA (breakdown product of dopamine) elevated in urine
4) Less likely to develop HTN
5) N-myc overexpression with rapid progression