CNS Tumors - Fremont-Smith Flashcards
low grade astrocytoma nutations
TP53
isocitrate DH
exception - pilocytic astrocytoma
loss of 22q
meningioma
1p and 19q codeletion
oligodencroglioma
good chemo response
isochromosome 17q
medulloblastoma
anaplastic astrocytoma-glioblastoma
EGFR amplification
PTEN alteration
NF1
17q
also with pheochromocytoma and GIST tumors
NF2
22q
vestibular and PN schwannoma, meningioma
tuberous sclerosis
TSC1/9q
TSC2/16p
renal AMLs, lung LAM, cardiac rhabdomyoma
li fraumeni syndrome
TP53/17p
astrocytoma
breast ca, bone and soft tissue sarcoma
bilateral optic nerve astrocytoma
neurofibromatosis type 1
von-recklinghausen
auto dom
chromosome 17q - neurofibromin protein
also - cafe au lait spots
primary CNS tumor
uncommon but fatal
multiple mets to brain
melanoma
grade 1 astrocytic tumor
pilocytic astrocytoma
grade 4 astrocytic tumor
glioblastoma
medulloblastoma
embryonal tumor
more in young kids cerebellum
-because more mitosis in young brain
primary CNS tumors of adults
meningioma 34% glioblastoma 16.7% pituitary 13.1% astroctoma 7% medulloblastoma 1%
meningioma
dura
-extra-axial
excision cures it
from meningothelial cells of arachnoid
more in women
genetic of meningioma
loss of chromosome 22q - LOF merlin
multiple meningioma
in NF2
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
