Pheochromocytoma

Question 1

what is pheochromocytoma?

Answer 1

catecholamine secreting adrenal tumor

Question 2

where are most pheochromocytoma located

Answer 2

98% are in abdomen

of those, 85% are in adrenals

Question 3

what do pheochromocytoma secrete?

Answer 3

norepinephrine > epinephrine > dopamine

Question 4

triggers for pheochromocytoma

Answer 4

pressure, meds, blood flow to tumor, surgery, exercise, pregnancy

Question 5

percentage of pheochromocytoma that are malignant

Answer 5

10%

Question 6

pheochromocytoma metastasis sites

Answer 6

bone, liver, lymph nodes

Question 7

pheochromocytoma can be associated with what genetic/familial syndromes?

Answer 7

MEN syndrome
von Hippel-lindau
neurofibromatosis

Question 8

4 cardinal physical exam findings to diagnose pheochromocytoma

Answer 8

1. HTN
2. Palpitations
3. Headaches
4. Excessive sweating

Question 9

for diagnosis of pheochromocytoma, a 24 hr urinary collection will show increased ____?

Answer 9

increased metanephrine and vanillylmandelic acid

Question 10

what imaging should be done to diagnose pheochromocytoma

Answer 10

MRI
CT
PET

Scintigraphy if labs indicate pheochromocytoma but above imagine doesn’t

Question 11

best treatment for pheochromocytoma

Answer 11

complete adrenalectomy (SURGERY)

Question 12

should you give an alpha or beta blockade first for treating pheochromocytoma

Answer 12

ALPHA- never beta first

Question 13

prognosis of pheochromocytoma

Answer 13

5 yr survival rate is 95%

malignant 5 year survival rate is less than 50%

Question 14

pheochromocytoma epidemiology

Answer 14

any age, peaks in 20-40 yrs
10% in kids
10% discovered incidentally
50% discovered during autopsy

Question 15

catecholamine effect of alpha adrenergic receptors

Answer 15

HTN, increased cardiac contractility, glycogenolysis, gluconeogenesis, intestinal relaxation

Question 16

catecholamine effect of beta adrenergic receptors

Answer 16

increased cardiac contractility, increased HR

Question 17

complications of pheochromocytoma

Answer 17

myocarditis, cardiomyopathy, pulmonary edema

if undiagnosed in pregnancy: mortality rate for mother is 48%, fetus is 55%