Pheo + paraganglioma Flashcards
1
Q
cell of origin of pheo / paraganglioma
A
sympathetic or parasympathetic tissue
2
Q
location of sympathetic PPGLs
A
adrenal (80%)
extra-adrenal abdominal locations (20%)
3
Q
location of parasympathetic PPGLs
A
thorax, head or neck
4
Q
what is secreted by sympathetic PPGLs
A
norepinephrine, epinephrine
5
Q
what is secreted by the parasympathetic PPGLs
A
rarely secrete catecholamines (only 4%)
6
Q
four germline mutations that predispose to the development of PPGLs
A
VHL, SDH A/B/C/D, NF1, RET
7
Q
how common are germline mutations in pediatric PPGLs?
A
80% - should have genetic testing
8
Q
risk factors for metastatic disease
A
- SDHB germline mutation
- extra-adrenal location
- large primary > 5cm
- younger age at diagnosis
- elevated 3-methoxytyramine levels
9
Q
diagnosis of PPGLs
A
- fractionated metanephrines in urine or plasma
- PET scan
- cross-sectional imaging
10
Q
clinical findings of PPGLs
A
- triad: headache, diaphoresis, tachycardia in setting of hypertension
- hypertension is the most common sign
- headache ~ 90%
