(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

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
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