CNS Tumors - Fremont-Smith Flashcards by Matt Stanton

low grade astrocytoma nutations

TP53
isocitrate DH

exception - pilocytic astrocytoma

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loss of 22q

meningioma

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1p and 19q codeletion

oligodencroglioma

good chemo response

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isochromosome 17q

medulloblastoma

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anaplastic astrocytoma-glioblastoma

EGFR amplification

PTEN alteration

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NF1

17q

also with pheochromocytoma and GIST tumors

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NF2

22q

vestibular and PN schwannoma, meningioma

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tuberous sclerosis

TSC1/9q
TSC2/16p

renal AMLs, lung LAM, cardiac rhabdomyoma

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li fraumeni syndrome

TP53/17p

astrocytoma

breast ca, bone and soft tissue sarcoma

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bilateral optic nerve astrocytoma

neurofibromatosis type 1
von-recklinghausen

auto dom

chromosome 17q - neurofibromin protein

also - cafe au lait spots

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primary CNS tumor

uncommon but fatal

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multiple mets to brain

melanoma

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grade 1 astrocytic tumor

pilocytic astrocytoma

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grade 4 astrocytic tumor

glioblastoma

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medulloblastoma

embryonal tumor

more in young kids cerebellum
-because more mitosis in young brain

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primary CNS tumors of adults

meningioma 34%
glioblastoma 16.7%
pituitary 13.1%
astroctoma 7%
medulloblastoma 1%

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meningioma

dura
-extra-axial

excision cures it

from meningothelial cells of arachnoid

more in women

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genetic of meningioma

loss of chromosome 22q - LOF merlin

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multiple meningioma

in NF2

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prognosis of meningioma

majority - good

-benign

post radiation

meningioma

hyalinzed whorls and calcified areas

meningioma

gliomas

astromcytomas, oligodendrogliomas, ependymomas

not from specific cell type
-arise from progenitor cell that differentiates down one cellular lineage

pilocytic astrocytoma

grade 1

most frequent glial tumor in children

well circumscribed - ring lesion

solid and cystic

good prognosis

cyst and solid lesion of brain

pilycytic astromcytoma recurrent - cyst comes back, solid does not

GFAP positive

tumor astrocytes - disorganized reactive astrocytes - gliosis - more organization

diffuse astrocytoma

grade 2 infiltrating seizures, HA, focal signs tendency to progress survival 5-7 years malignant cells

glioblastoma

has necrosis

glioblastoma

grade 4 most frequent glial tumor in adults malignant seizures, HA, focal deficits supratentorial survival - 1 year

primary vs. secondary glioblastoma

primary - loss of heterozygosity at 10q -right to glioblastoma - 95% cases secondary - progress from low grade - IDH1 and IDH2 point mutations - better prognosis - grow slower

imaging of glioblastoma

leaky vessels | -contrast to viable areas

supratentorial, cortical, and white matter

oligodendroglioma majority frontal

oligodendroglioma

frontal lobe 40-50yo HA and seizures often calcified codeletion - 1p and 19q - good response to chemo

good response to chemo

oligodendroglioma with codeletion 1p and 19q

fried egg and GFAP with calcifications histo

oligodendroglioma

medulloblastoma

malignant - in children posterior fossa - majority in vermis disseminates along SA space 50-70% 5 year prognosis

external granular layer of cerebellum

medulloblastoma small blue cell tumor

homer wright rosettes

medulloblastoma group of tumor cell around a fibrillary center

prognosis medulloblastoma

highly malignant leptomeningeal dissemination CSF - high protein and low glucose - with tumor cells -CSF cytology - to monitor spread of tumor

thick layer of tumor around spinal cord - or matted together cauda equina

medulloblastoma

primary CNS lymphoma

frequent in AIDS - but not anymore digguse large B cell lymphoma immunocompromised - EBV positive

corticosteroids

cause apoptosis of primary CNS lymphomas

mets to brain

majority of brain tumors men - carcinoma of lung women - breast carcinoma

highest rate of mets to bain

melanoma

meningeal carcinomatosis

tumor to virchow robins space high protein CSF low glucose CSF few lymphocytes CSF

ependymoma

slow growing tumor of children and young adults hydrocephalus intraventricular or on spinal cord

bilateral schwannoma

NF2 benign 40-60yo

schwannoma

more often in peripheral nerves but in CNS is vestibular branch of CN VIII