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Neuro weeks4-6 - Keith > 31b - Pediatric Brain Tumors > Flashcards

31b - Pediatric Brain Tumors Flashcards

1
Q

Where is the most common site for PED tumors: supratentoria, infretentorial, or midline?

A

infratentorial most common in PEDs.

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2
Q

What are supratentorial peds tumors?

A

suprasellar (chiasmal glioma, craniopharyngioma)
hemispheric (astrocytoma)
pineal

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3
Q

What are infratentorial tumors in peds?

A

Most common are cerebellum and 4th ventricle

>posterior fossa (medulloblast, astrocy, ependy, and glioma)

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4
Q

What is most common pediatric solid tumor?

A

brain tumor

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5
Q

What is survival rate for brain tumors in 1st decade?

A

65%

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6
Q

What is most common Peds tumor?

A

astrocytoma>PNET>gliomas>ependymomas

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7
Q

What are Cowden (xPTEN) syndromes risks for?

A

high mTOR. dysplastic gangliocytoma in cerebellum

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8
Q

What are nevoid basal cell carcinoma/gorlin syndrome risks for?

A

medulloblastoma (ch. 9, PTCH mutation)

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9
Q

What is an APC gene mutation a risk for?

A

turcot syndrome, medulloblastoma and GBM

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10
Q

What is likely location of a tumor causing localized seizures and hemiparesis?

A

supratentorial

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11
Q

What symptoms would you expect for a midline tumor?

A

endocrinopathies, hydrocephalus (early AM headache with n/v, bulging fontanelle, sundowning eyes, CN6 palsy)

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12
Q

What are signs of a infratentorial tumor?

A

high ICP due to compression of 4th ventrical.

papilledema w/blurring of optic disk

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13
Q

What are signs of brainstem tumor?

A 
diplopia, dysarthria, dysphagia.
crossed weakness (lesion rostral to pyramidal decussation) = ipsilateral CN function and contralateral UMN findings.
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14
Q

What is JPA? Where is it commonly olcated?

A

juvenile pilocystic astrocytoma (grade 1).

usually supra or infratentorial, most on posterior fossa.

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15
Q

What can JPA progress to?

A

GBM

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16
Q

What is pathogenesis of JPA?

A

mutation of BRAF (fusion or v600e) and NF1 cause oncogenic process of MAPK path.
mtuations activate RAF and MAPK pathways that incrase nuclear transcription.

17
Q

Where are BRAF fusion mutation tumors common?

BRAF v600e?

A 
fusion = cerebellum
v600e = extracerebellum
18
Q

Whhere are NF1 mutation types of JPA common?

A

optic pathways

19
Q

What is gross appearance of JPA?

A

cYSTIC.

20
Q

what is microscopic pathology of JPA?

A

pilocytic, hair like projections off neoplastic cells ROSENTHAL FIBERS that are GFAP+.
Hyalinization of blood vessels

21
Q

What is prognosis for JPA?

A

good, it is low grade

22
Q

What is medulloblastoma?

A

a highly malignant primitive tumor of the CEREBELLUM.

23
Q

What is PNET?

A

highly malignant primitive tumor of the CEREBRUM

24
Q

What are histochemical markers for medulloblastoma and PNET?

A

TrkC, Erb, C-myc, neurotrophinin

25
Q

What is pathogenesis of medulloblastoma?

A

SHH and Wnt pathways.
inactive SHH has high Ptch inhibition of smooth cilium transfer so high degradation of smoothened and SufU-Gli2/3

Active SHH has low PTCH inhibition so active smoothened to cilium transfer so decreased SuFu mediated gli2 degradation adn increased gli2 transcription

Inactive WNT has inactive frizzed receptors and increased B-catenin phosphorylation/degradation

Active Wnt has active Frizzled receptors and high B-catenin to nucleus transcription

26
Q

What is best prognosis for medulloblastoma?

A

wnt > smoothened/PTCH > c-myc (bad)

27
Q

Why is medulloblatoma difficult for resection?

A

it spready via CSF to other structures

28
Q

What type of tumor has round, blue carrot shaped cells with Homer wright rossette?

A

medulloblastoma

29
Q

Is medulloblastoma sensitive to radiotherapy?

A

you betcha

30
Q

What is a brainstem glioma?

A

subgroup of astrocytoma.
diffuse intrinsic pontine glioma.
Terrible prognosis.

31
Q

What is gross pathology/MRI finding for brainstem glioma?

A

tumor that engulfs tha basilar artery

32
Q

What is clinical presentation for brainstem glioma?

A

classic triad: CST long tract sings of weak/hemiparesis, ataxia, and CN6,7,8 signs.

33
Q

What is ependymoma?

A

tumor of ependymal lined ventricles

34
Q

What is the PNET variant of ependymoma?

A

ependymoblastoma

35
Q

Tumor is found growing into ventricular system, histo shows perivascualr psuedorossettes. what do you suspect

A

ependymoma

36
Q

What is micropath for ependymoma?

A

perivascular pseudorossettes

37
Q

Which peds tumor can you NOT perform surgery on?

A

brain stem glioma

38
Q

How long should you try to hold off on radiation therapy in kids?

A

until at least 3, best if after 7 so they can develop. Will have defecits if treated early.

Neuro weeks4-6 - Keith (37 decks)

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