(31B) Pediatric Brain Tumors Flashcards

1
Q

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

A

infratentorial

supratentorial are more common in 1st year

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2
Q

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

A

M

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3
Q

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

A

astrocytomas > PNETs > gliomas > ependymomas

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4
Q

What are the most common anatomical sites of pediatric CNS tumors

A

cerebellum > cerebrum and brain stem

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5
Q

what tumors are most common in children < 1 yo

A

gliiomas
teratomas
PNET
Chorid plexus

**supratentorial

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6
Q

what tumors are most common in children 1 - 11 yo

A

medulloblastoma
ependymoma
brain stem glioma

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7
Q

what tumors are most common in children > 11 yo

A

gliomas
PNET/medulloblastoma
germ cell

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8
Q

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

A

wiskott-aldrich syndrome
ataxia-telangiectasi
aquired immunodeficiency

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9
Q

need to know genetic syndromes?

A

?

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10
Q

what are the glial tumors common in kids

A
astocytomas
oligiodendrogliomas
ependymal tumors
choroid plexus tumors
mixed gliomas 
GBM
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11
Q

what are the neuronal tumors common in kids

A

gangliocytomas

anaplastic ganglioma

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12
Q

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

A

PNET
PNET with differentation
medulloepithelioma

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13
Q

what are the pineal gland tumors common in kids

A

pineocytoma

pinealblastoma

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14
Q

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

A

midline–hypothalamic, optic, pineal, craniophyngioma

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15
Q

what would the clinical manifestations be of a supratentorial tumor

A

seizures, hemiparesis

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16
Q

what would the clinical manifestations be of a infratentorial tumor

A

signs/sympotms related to inc ICP

17
Q

what would the clinical manifestations be of a brain stem tumor

A

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

18
Q

___ is assc with 80% of midline brain tumors

A

hydrocephalus

19
Q

ICP triad

A

AM headaches, N/V, lethargy

20
Q

how long to S&S present before diagnosis of ICP

A

4-6 mos

21
Q

most common childhood CNS tumor

A

low grade astrocytoma

22
Q

grade ___ astocytomas are malignant

A

III (anaplastic astrocytoma) and IV (glioblastoma multiforme)

23
Q

characteristic histo and common histo feature of juvenile pilocytic astrocytoma

A

characteristic =eosinophillic rosenthal fibers

common = hyaliinization of blood vessels

24
Q

mutation seen in juvenile pilocytic astrocytoma

A

activation of MAPK and RAF pathways via

  1. BRAF mutation or 2. fusion
  2. NF1 mutation leading to KRAS activation
25
Q

medulloblastoma arise from what cells

A

primitive nerve cells

26
Q

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

A

cerebrum = PNET

cerebellum = medulloblastoma

27
Q

how does medulloblastoma typically spread

A

via CSF

28
Q

characteristc histo seen with medulloblastoma

A

homer-wright rossettes

29
Q

signaling pathway that is active in medulloblastoma

A

Shh and Wnt

30
Q

what symptomas are consistent with diagnosis of diffuse intrinsic pontine glioma

A

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

31
Q

characteristic MRI finding consistent with diagnosis of diffuse intrinsic pontine glioma

A

tumor engulfs basilar artery with diffuse extension into pons

32
Q

where are ependymoma tumors most characteristically found?

A

posterior cranial fossa > spinal cord

33
Q

characteristic histo seen with ependymoma tumors

A

perivascular pseudo rosettes that form a central lumen

34
Q

PNET variant of ependymoma is called

A

ependymoblastoma

35
Q

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

A

surgery–diffuse brain stem gliomas

36
Q

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

A

3 but 7 is better