Pheochromocytoma

Question 1

Pheochromocytomas are rare tumors that commonly occur in the _______ ________.

Answer 1

Adrenal Medulla

Question 2

A pheo in the adrenal medulla will mostly secrete_______ but also __________.

Answer 2

Mostly Norepinephrine but also epinephrine

Question 3

Peak incidence of pheo’s is what age range?

Answer 3

30s -40s

Question 4

The 3 familial autosomal dominant disorders associated c pheo’s

Answer 4

MEN2
Von-hippel-Lindau disease (VHL)
Neruofibromatosis Type 1 (NF1)

Question 5

Cells that pheo’s arise from in the adrenal medulla

Answer 5

Chromaffin cells

Question 6

Symptoms are caused by excessive catecholamines that stimulate _______ and _______ receptors

Answer 6

Alpha and Beta

Question 7

Classic Triad of pheo

Answer 7

Headache
sweating
tachycardia (palpitations)

Question 8

T or F: Sustained or paroxysmal HTN is the most common sign.

Answer 8

True

Question 9

A typical spell typically will last __ to ___ min

Answer 9

10-15min

Question 10

T or F: Pheo spells mimic anxiety reactions

Answer 10

true

Question 11

Crisis could lead to…(8 things)

Answer 11

ARDS
acute heart failure
Pulmonary edema
Severe hypotension
Kidney failure
Liver failure
ICH
Death

Question 12

T or F: Early onset HTN (<20 years) does not need to be tested for pheo

Answer 12

False. Early onset
Resistant HTN
HTN with new onset or atypical diabetes (in slender person) should all be tested

Question 13

1st Step of Dx of pheo

Answer 13

Confirm catecholamine oversecretion
-24hr urine fractionated metanephrines and catecholamines
OR
-Plasma fractionated metanephrines

Question 14

2nd step of Dx of pheo

Answer 14

Identify tumor on imaging if + for hypersecretion

CT or MRI

Question 15

T or F: If a patients with “spells” tests are normal and show no oversecretion there is no need for further testing.

Answer 15

False. If they have spells you shoiuld repeats tests during a spell

Question 16

T or F: Treatment for patients with pheo should be immediate surgical resection without any preparatory medications.

Answer 16

False.
Preoperative stabilization should be done:
-Alpha blockers + phenoxybenzamine
-Beta blockers + Liberal salt /fluid intact for 2-3 days
-may also add calcium channel blockers

Question 17

T or F: For preoperative stabilization you should start Beta blockers first then add in alpha blockers later.

Answer 17

False. Never give B blockers alone.
Avoid Beta blockers for several days until adequate alpha blockade has been established (to avoid HTN crisis from unopposed alpha stim)

Question 18

T or F: Post surgery patients need 5 years of follow up.

Answer 18

False. Lifetime follow up needed

Metastases might become apparent years later

Question 19

BP and plasma metanephrines should be checked ___ to ___weeks post op and every ___months for at least 5 years, then yearly for life.

Answer 19

4-6 weeks post op
every 6 months for 5 years
then yearly for life