1
Q
Risk groups
A
2
Q
Molecular sub groups
A
Wingless (WNT)
Sonic hedgehog
Group 3
Group 4
3
Q
Incidence
A
<19 yeard old
M > F
4
Q
5 year OS
A
65-70%
5
Q
Poor prognostic factors
A
Age <3 years
Metastatic tumor
GTR vs STR
molecular subtypes
6
Q
Risk Factors
A
Li-Fraumeni syndrome
Turcot syndrome
Gorlin syndrome
Germline mutations
7
Q
Common germline mutations
A
BRAC2
PALB2
GPR161
ELP1
CREBBP
EP300
8
Q
Li-Fraumeni syndrome
A
- Germline TP53 mutation
breast cancer
sarcoma
leukemia
9
Q
Turcot syndrome
A
- Germline APC/FAP mutation
colon cancer, high grade glioma and medulloblastoma
10
Q
Gorlin syndrome
A
- Germline PTCH1 and SUFU mutation
multiple BCC, skeletal abnormalities
11
Q
Clinical presentation
A
headaches
nausea
vomiting
ataxia
12
Q
Treatment paradigm
A
Maximum safe resection
RT
Chemotherapy
13
Q
A
Pediatrics
flashcards
Decks in class (11)
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