31B Peds Brain Tumors

Question 1

Epidemiology of pediatric brain tumors?

Answer 1

MC 1st decade, M>F

Question 2

MC type of brain tumor in peds?

Answer 2

Astrocytomas > PNETs > gliomas > ependymomas

Question 3

Tumors that occur almost exclusively in kids?

Answer 3

medulloblastoma
supratentorial PNET
pineoblastomas

Question 4

MC location of brain tumor in peds?

Answer 4

infratentorial > supratentorial > midline

Question 5

MC brain tumor in peds <1?

Answer 5

supratentorial (gliomas, teratomas, PNET, choroid plexus)

Question 6

MC brain tumor in peds 1-11?

Answer 6

infratentorial > supratentorial

medulloblastoma, ependymoma, brain stem glioma

Question 7

MC brain tumor in peds >11?

Answer 7

infratentorial = supratentorial

gliomas, medulloblastomas, PNET, germ cell

Question 8

Pre-disposing factors for brain stems?

Answer 8

ionizing radiation
other tumors
immune suppression
family hx

Question 9

Neurofibromatosis I predisposes to:

Answer 9

optic gliomas, meningiomas, ependymomas

Question 10

Neurofibromatosis II predisposes to:

Answer 10

bil vestibular schwannomas

Question 11

Tuberous sclerosis predisposes to:

Answer 11

subependymal giant cell tumors

Question 12

Von Hippel Lindau predisposes to:

Answer 12

hemangioblastomaa

Question 13

Turcot syndrome predisposes to:

Answer 13

medulloblastomas, glioblastoma multiforme

Question 14

Cowden predisposes to:

Answer 14

dysplastic gangliocytoma of cerebellum

Question 15

Li-Fraumenti predisposes to:

Answer 15

astrocytoma, medulloblastoma

Question 16

Nevoid basal cell (Gorlin) predisposes to:

Answer 16

medulloblastoma

Question 17

Hydrocephalus is associated with 80% of:

Answer 17

midline tumors

Question 18

Hydrocephalus initial symptoms:

Answer 18

early AM intermittent HA + NV (*ICP)

Question 19

MC childhood brain tumor?

Answer 19

astrocytoma (benign, low grade)

Question 20

Prognosis of astrocytoma?

Answer 20

> 75% 5yr survival with complete resection

pilocytic >90% 5yr survival

Question 21

What pathway is activated in pilocytic astrocytoma?

Answer 21

loss of NF1 = KRAS active
–>BRAF or v600E mutations
(mutations have a particular location and progonsis)

Question 22

Epidemiology for medulloblastoma?

Answer 22

3-4y, M>F

Question 23

Prognostic factors for medulloblastoma?

Answer 23

• age of the patient
• histology of the disease
• extent of disease (i.e sp cd mets or LMD)
• extent of residual disease (how much resected)
• various molecular markers

Question 24

Prognosis of medulloblastoma?

Answer 24

malignant and infiltrating, preventing complete resection (srx + RT)

Question 25

Histo of medulloblastoma?

Answer 25

- Densely cellular - Round, oval or angulated (‘carrot-shaped’) - Low vascular density - Homer-Wright rosette (tumor cells surrounding fibrillar area
) - Hyperchromatic


Question 26

Trx for brainstem glioma?

Answer 26

srx CI, RT for temporary improvement; supportive care

Question 27

Prognosis for brainstem glioma?

Answer 27

BAD, <2 years

Question 28

Diffuse intrinsic pontine glioma: | Prognosis?

Answer 28

bad--medium survival 9-11mo

Question 29

Diffuse intrinsic pontine glioma: | Epidemiology?

Answer 29

7yo

Question 30

Diffuse intrinsic pontine glioma: | MRI findings?

Answer 30

tumor engulfs basilar artery, extends into pons

Question 31

Diffuse intrinsic pontine glioma: | Symptoms?

Answer 31

corticospinal tract = weakness or hemiparesis, ataxia, CN 6/7/8

Question 32

Epidemiology of ependymoma?

Answer 32

0-7 y/o, M=F

Question 33

Origin of ependymoma?

Answer 33

lining of ventricles or central canal of spinal cord

Question 34

MC location of ependymoma?

Answer 34

posterior fossa >> spinal cord

Question 35

Histo of ependymoma?

Answer 35

- perivascular pseudorosettes of glial tumor cells, radially arranged around blood vessels - true ependymal rosettes of tumor cells that form a central lumen on their own

Question 36

Gross path of ependymoma?

Answer 36

low grade, locally invasive

Question 37

PNET variant of ependymoma?

Answer 37

ependymoblastoma

Question 38

Prognostic factors of ependymoma?

Answer 38

srx resection brain stem involvement age stage at presentation

Question 39

Trx of ependymoma?

Answer 39

1. srx (goal = dx and decr tumor burden) 2. RT (varies according to histo) 3. chemo

Question 40

Tumors treated w RT?

Answer 40

``` high/low grade astrocytoma medulloblastoma/PNET ependymoma germ cell craniopharyngioma brain stem glioma ```

Question 41

Effects of RT?

Answer 41

intellectual impairments memory deficits inability to acquire new knowledge

Question 42

Cognitive impairments following RT are most pronounced in pts...

Answer 42

less than 4-7 y/o

Question 43

Tumors treated w chemo?

Answer 43

- int/high-risk medulloblastoma - high-grade glioma, PNET, ATRT - germ cell - low grade astrocytoma