Brain Tumours

Question 1

?most common brain tumour of childhood
From which tissue
Where do they arise
What prop of kids have mets at pres

Answer 1

Medulloblastoma 20% of CNS tumours
Highly malignant primitive neuroectodermal
Arise from cerebellum (vermis typically)
CNS dissemination risk
Mets in up to 1/3 at pres
65%males
Peak age 7
Chang classification M0-5

Question 2

What mutation is associated with ATRT

Answer 2

Atypical teratoid rhabdoid tumours
INI1 (hSNF5) mutation or loss
Highly malignant, usually

Question 3

Types of MB and prog

Answer 3

30% Sonic hedgehog 75% 5y surv
10% WNT “is mint” 95% 5y surv
25% Group 3 DO WORST 40-60% 5y surv
25% group 4 75% 5y surv

Question 4

Most common low grade glioma in kids

Answer 4

JPA
Juvenile pilocytic astrocytoma (grade 1) indolent
2/3 in posterior fossa
Better prog in NF-1 kids
Surgery +/- RT +/- CT

Question 5

What molecular markers are found in MB

Answer 5

C-Myc over expression

TrK-C over expression