(31B) Pediatric Brain Tumors Flashcards Preview

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Flashcards in (31B) Pediatric Brain Tumors Deck (36):
1

___ tumors are most common in children (except in 1st year of life)

infratentorial

(supratentorial are more common in 1st year)

2

Brain tumors have M or F prodominane in 1st decade of life

M

3

What are the 3 most common CNS tumors affecting kids in greatest to least freq

astrocytomas > PNETs > gliomas > ependymomas

4

What are the most common anatomical sites of pediatric CNS tumors

cerebellum > cerebrum and brain stem

5

what tumors are most common in children < 1 yo

gliiomas
teratomas
PNET
Chorid plexus

**supratentorial

6

what tumors are most common in children 1 - 11 yo

medulloblastoma
ependymoma
brain stem glioma

7

what tumors are most common in children > 11 yo

gliomas
PNET/medulloblastoma
germ cell

8

what are syndromes that cause immunosuppression in children (therefore leaving them susceptible to tumor development)

wiskott-aldrich syndrome
ataxia-telangiectasi
aquired immunodeficiency

9

need to know genetic syndromes?

?

10

what are the glial tumors common in kids

astocytomas
oligiodendrogliomas
ependymal tumors
choroid plexus tumors
mixed gliomas
GBM

11

what are the neuronal tumors common in kids

gangliocytomas
anaplastic ganglioma

12

what are the primitive neuroectodermal tumors (PNET) common in kids

PNET
PNET with differentation
medulloepithelioma

13

what are the pineal gland tumors common in kids

pineocytoma
pinealblastoma

14

based on the following manifestations where would you expect the CNS tumor to be found?

endocrinopathies such as DI, growth disorders
decreased vision and visual field defecits

midline--hypothalamic, optic, pineal, craniophyngioma

15

what would the clinical manifestations be of a supratentorial tumor

seizures, hemiparesis

16

what would the clinical manifestations be of a infratentorial tumor

signs/sympotms related to inc ICP

17

what would the clinical manifestations be of a brain stem tumor

CN deficits: double vision, slurred speech, swallowing disorders, and "crossed weakness"

18

___ is assc with 80% of midline brain tumors

hydrocephalus

19

ICP triad

AM headaches, N/V, lethargy

20

how long to S&S present before diagnosis of ICP

4-6 mos

21

most common childhood CNS tumor

low grade astrocytoma

22

grade ___ astocytomas are malignant

III (anaplastic astrocytoma) and IV (glioblastoma multiforme)

23

characteristic histo and common histo feature of juvenile pilocytic astrocytoma

characteristic =eosinophillic rosenthal fibers

common = hyaliinization of blood vessels

24

mutation seen in juvenile pilocytic astrocytoma

activation of MAPK and RAF pathways via
1. BRAF mutation or 2. fusion
3. NF1 mutation leading to KRAS activation

25

medulloblastoma arise from what cells

primitive nerve cells

26

what are medullblastomas called when they are found in cerebrum vs cerebellulum

cerebrum = PNET

cerebellum = medulloblastoma

27

how does medulloblastoma typically spread

via CSF

28

characteristc histo seen with medulloblastoma

homer-wright rossettes

29

signaling pathway that is active in medulloblastoma

Shh and Wnt

30

what symptomas are consistent with diagnosis of diffuse intrinsic pontine glioma

weakness or hemiparesis (cortical spinal tract signs)
ataxia
CN 6. 7. 8 defecits

31

characteristic MRI finding consistent with diagnosis of diffuse intrinsic pontine glioma

tumor engulfs basilar artery with diffuse extension into pons

32

where are ependymoma tumors most characteristically found?

posterior cranial fossa > spinal cord

33

characteristic histo seen with ependymoma tumors

perivascular pseudo rosettes that form a central lumen

34

PNET variant of ependymoma is called

ependymoblastoma

35

what is the preferred modality of treatment for pediatric CNS tumors? what is the exception

surgery--diffuse brain stem gliomas

36

bc the brain is rapidly developing RT they try to delay RT until what age?

3 but 7 is better