Brain Tumours

Question 1

Median age for medulloblastoma?

Answer 1

5-7yrs

Question 2

Most common position for medulloblastoma?

Answer 2

Cerebellar vermis

Commonly aise from roof of 4th ventricle

Question 3

Most highly malignant Paediatric Brain tumour?

Answer 3

Medulloblastoma

can met to bone/BM

Question 4

What brain tumour do kids with NF1 get?

Answer 4

low grade glioma (optic pathway)

Question 5

What brain tumour do kids with TS get?

Answer 5

giant cell astrocytoma (close to ventricular system)

Question 6

If you see a patient with medulobastoma + skin pigmentation what should you think of?

Answer 6

GORLIN syndrome

PTCH gene anomaly

Question 7

Hemangioblastoma in posterior fossa.

What condition should you think of?

Answer 7

Von Hippel Lindau

Question 8

Choroid plexus carcinoma.

What condition should you consider?

Answer 8

Li Fraumani syndrome

Question 9

General age cut off for XRT?

Answer 9

<3yrs

Question 10

What is the tentorium?

Answer 10

Layer of meninges which separates the brainstem from the cortex

Question 11

Where is the most common site for brain tumours in children?

Answer 11

Infratentorium

Question 12

Supracellar tumours occur where?

Answer 12

thalamus/hypothalami

Question 13

Function of astrocytes in brain?

Answer 13

provide metabolic connection between blood vessels and neurons

Question 14

Function of oligodendrocytes in brain?

Answer 14

myelination in CNS

Question 15

What does PNETS stand for?

Answer 15

Primitive Neuroectodermal tumour

Question 16

What is the most common type of PNET tumour in children?

Answer 16

Medulloblastoma

Question 17

Which Medulloblastoma has best prognosis?

Answer 17

medulloblastoma with extensive nodularity

Question 18

Which medulloblastoma has least favourable prognosis?

Answer 18

large cell/anaplastic

Question 19

Which two genetic pathways should you be aware of in medulloblastoma?

Answer 19

SHH: Sonic hedgehog pathway (less favourbale)
Wnt: Wint pathway (good prognosis, ?nil XRT)

Question 20

WHO grading system for gliomas (or astrocytomas)?

Answer 20

1. Pilocytic astrocytoma
2. Fibrillary astrocytoma
3. Anaplastic astrocytoma
4. Glioblastoma Multiforme

Question 21

What is the prognosis for diffuse intrinsic pontine glioma?

Answer 21

Bad

almost 100% mortality within 2yrs

Question 22

If you see a calcified mass with cysts + hemorrhage at the back of the brain in child <5 what is the most likely diagnosis?

Answer 22

ependymoma

Question 23

What is important to recall about germinomas?

Answer 23

Germinomas Don’t secrete hormones

Question 24

What is a high B-HCG characteristic of?

Answer 24

choriocarcinoma

Question 25

What is a high alpha-FP characteristic of?

Answer 25

yolk sac tumours