(31B) Pediatric Brain Tumors Flashcards
___ tumors are most common in children (except in 1st year of life)
infratentorial
supratentorial are more common in 1st year
Brain tumors have M or F prodominane in 1st decade of life
M
What are the 3 most common CNS tumors affecting kids in greatest to least freq
astrocytomas > PNETs > gliomas > ependymomas
What are the most common anatomical sites of pediatric CNS tumors
cerebellum > cerebrum and brain stem
what tumors are most common in children < 1 yo
gliiomas
teratomas
PNET
Chorid plexus
**supratentorial
what tumors are most common in children 1 - 11 yo
medulloblastoma
ependymoma
brain stem glioma
what tumors are most common in children > 11 yo
gliomas
PNET/medulloblastoma
germ cell
what are syndromes that cause immunosuppression in children (therefore leaving them susceptible to tumor development)
wiskott-aldrich syndrome
ataxia-telangiectasi
aquired immunodeficiency
need to know genetic syndromes?
?
what are the glial tumors common in kids
astocytomas oligiodendrogliomas ependymal tumors choroid plexus tumors mixed gliomas GBM
what are the neuronal tumors common in kids
gangliocytomas
anaplastic ganglioma
what are the primitive neuroectodermal tumors (PNET) common in kids
PNET
PNET with differentation
medulloepithelioma
what are the pineal gland tumors common in kids
pineocytoma
pinealblastoma
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
what would the clinical manifestations be of a supratentorial tumor
seizures, hemiparesis
what would the clinical manifestations be of a infratentorial tumor
signs/sympotms related to inc ICP
what would the clinical manifestations be of a brain stem tumor
CN deficits: double vision, slurred speech, swallowing disorders, and “crossed weakness”
___ is assc with 80% of midline brain tumors
hydrocephalus
ICP triad
AM headaches, N/V, lethargy
how long to S&S present before diagnosis of ICP
4-6 mos
most common childhood CNS tumor
low grade astrocytoma
grade ___ astocytomas are malignant
III (anaplastic astrocytoma) and IV (glioblastoma multiforme)
characteristic histo and common histo feature of juvenile pilocytic astrocytoma
characteristic =eosinophillic rosenthal fibers
common = hyaliinization of blood vessels
mutation seen in juvenile pilocytic astrocytoma
activation of MAPK and RAF pathways via
- BRAF mutation or 2. fusion
- NF1 mutation leading to KRAS activation
medulloblastoma arise from what cells
primitive nerve cells
what are medullblastomas called when they are found in cerebrum vs cerebellulum
cerebrum = PNET
cerebellum = medulloblastoma
how does medulloblastoma typically spread
via CSF
characteristc histo seen with medulloblastoma
homer-wright rossettes
signaling pathway that is active in medulloblastoma
Shh and Wnt
what symptomas are consistent with diagnosis of diffuse intrinsic pontine glioma
weakness or hemiparesis (cortical spinal tract signs)
ataxia
CN 6. 7. 8 defecits
characteristic MRI finding consistent with diagnosis of diffuse intrinsic pontine glioma
tumor engulfs basilar artery with diffuse extension into pons
where are ependymoma tumors most characteristically found?
posterior cranial fossa > spinal cord
characteristic histo seen with ependymoma tumors
perivascular pseudo rosettes that form a central lumen
PNET variant of ependymoma is called
ependymoblastoma
what is the preferred modality of treatment for pediatric CNS tumors? what is the exception
surgery–diffuse brain stem gliomas
bc the brain is rapidly developing RT they try to delay RT until what age?
3 but 7 is better
