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Neuro II > 31B Peds Brain Tumors > Flashcards

31B Peds Brain Tumors Flashcards

1
Q

Epidemiology of pediatric brain tumors?

A

MC 1st decade, M>F

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2
Q

MC type of brain tumor in peds?

A

Astrocytomas > PNETs > gliomas > ependymomas

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3
Q

Tumors that occur almost exclusively in kids?

A

medulloblastoma
supratentorial PNET
pineoblastomas

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4
Q

MC location of brain tumor in peds?

A

infratentorial > supratentorial > midline

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5
Q

MC brain tumor in peds <1?

A

supratentorial (gliomas, teratomas, PNET, choroid plexus)

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6
Q

MC brain tumor in peds 1-11?

A

infratentorial > supratentorial

medulloblastoma, ependymoma, brain stem glioma

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7
Q

MC brain tumor in peds >11?

A

infratentorial = supratentorial

gliomas, medulloblastomas, PNET, germ cell

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8
Q

Pre-disposing factors for brain stems?

A

ionizing radiation
other tumors
immune suppression
family hx

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9
Q

Neurofibromatosis I predisposes to:

A

optic gliomas, meningiomas, ependymomas

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10
Q

Neurofibromatosis II predisposes to:

A

bil vestibular schwannomas

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11
Q

Tuberous sclerosis predisposes to:

A

subependymal giant cell tumors

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12
Q

Von Hippel Lindau predisposes to:

A

hemangioblastomaa

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13
Q

Turcot syndrome predisposes to:

A

medulloblastomas, glioblastoma multiforme

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14
Q

Cowden predisposes to:

A

dysplastic gangliocytoma of cerebellum

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15
Q

Li-Fraumenti predisposes to:

A

astrocytoma, medulloblastoma

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16
Q

Nevoid basal cell (Gorlin) predisposes to:

A

medulloblastoma

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17
Q

Hydrocephalus is associated with 80% of:

A

midline tumors

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18
Q

Hydrocephalus initial symptoms:

A

early AM intermittent HA + NV (*ICP)

19
Q

MC childhood brain tumor?

A

astrocytoma (benign, low grade)

20
Q

Prognosis of astrocytoma?

A

> 75% 5yr survival with complete resection

pilocytic >90% 5yr survival

21
Q

What pathway is activated in pilocytic astrocytoma?

A

loss of NF1 = KRAS active
–>BRAF or v600E mutations
(mutations have a particular location and progonsis)

22
Q

Epidemiology for medulloblastoma?

23
Q

Prognostic factors for medulloblastoma?

A
  • age of the patient
  • histology of the disease
  • extent of disease (i.e sp cd mets or LMD)
  • extent of residual disease (how much resected)
  • various molecular markers
24
Q

Prognosis of medulloblastoma?

A

malignant and infiltrating, preventing complete resection (srx + RT)

25
Q

Histo of medulloblastoma?

A
  • Densely cellular
  • Round, oval or angulated (‘carrot-shaped’)
  • Low vascular density
  • Homer-Wright rosette (tumor cells surrounding fibrillar area
)
  • Hyperchromatic

26
Q

Trx for brainstem glioma?

A

srx CI, RT for temporary improvement; supportive care

27
Q

Prognosis for brainstem glioma?

A

BAD, <2 years

28
Q

Diffuse intrinsic pontine glioma:

Prognosis?

A

bad–medium survival 9-11mo

29
Q

Diffuse intrinsic pontine glioma:

Epidemiology?

30
Q

Diffuse intrinsic pontine glioma:

MRI findings?

A

tumor engulfs basilar artery, extends into pons

31
Q

Diffuse intrinsic pontine glioma:

Symptoms?

A

corticospinal tract = weakness or hemiparesis, ataxia, CN 6/7/8

32
Q

Epidemiology of ependymoma?

A

0-7 y/o, M=F

33
Q

Origin of ependymoma?

A

lining of ventricles or central canal of spinal cord

34
Q

MC location of ependymoma?

A

posterior fossa&raquo_space; spinal cord

35
Q

Histo of ependymoma?

A
  • perivascular pseudorosettes of glial tumor cells, radially arranged around blood vessels
  • true ependymal rosettes of tumor cells that form a central lumen on their own
36
Q

Gross path of ependymoma?

A

low grade, locally invasive

37
Q

PNET variant of ependymoma?

A

ependymoblastoma

38
Q

Prognostic factors of ependymoma?

A

srx resection
brain stem involvement
age
stage at presentation

39
Q

Trx of ependymoma?

A
  1. srx (goal = dx and decr tumor burden)
  2. RT (varies according to histo)
  3. chemo
40
Q

Tumors treated w RT?

A 
high/low grade astrocytoma
medulloblastoma/PNET
ependymoma
germ cell
craniopharyngioma
brain stem glioma
41
Q

Effects of RT?

A

intellectual impairments
memory deficits
inability to acquire new knowledge

42
Q

Cognitive impairments following RT are most pronounced in pts…

A

less than 4-7 y/o

43
Q

Tumors treated w chemo?

A
  • int/high-risk medulloblastoma
  • high-grade glioma, PNET, ATRT
  • germ cell
  • low grade astrocytoma

Neuro II (18 decks)

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