brainonco Flashcards
glioblastoma
grade 4 ,1 year median survival ,found in cerebral hemispheres ,can cross corpus callosum ,butterfly glioma
ass with EGFR amplification
astrocytes origin GFAP ,psuedopalisading pleomorphic tumor cells
border central areas of necrosis ,hmrg ,and microvascular proliferation
oligodendroglioma
relatively rare slow growing most often on frontal lobes
oligodendrocyte origin
fried egg cells round nuceli with clear cytoplasm
chicken wire capillary pattern
meningioma
most often near parasagittal region
ectra axial may have a dural attachment often asymptomatic ,resection
archanoid cell origin spindle cells
concentrically arranged in a whorled pattern
psommoma bodies
hemangioblastoma
most often cerebellar , von hippel lindau syndrome when found with retinal aniomas , can produce erythropoietin ,2ry polycythemia
blood vessel origin , closely arranged thin walled capillaries with minimal intervening parenchyma
pit adenoma
may be non functioning silent or hyperfunctioning hormone producing
non functional present with mass effect ,bitemporal hemianopia ,due to pressure on optic chiasm ,pituitary apoplexy ,
prolactinoma ,
dopamine agonist ,brompocriptine ,cabergoline , transsphenoidal resection
most commonly hyperplasia of lactotrophes
schwannoma
classically at the cerebellopontine angle
cn 5 7 and 8
but can be along 8 internal acoustic meatus
bilateral nf2
schwann cell origin s100
biphasic dense hypercellular areas containing spindle cells alternating with hypocellular myxoid areas
osmotic demyelination syndrome
also called central pontine myelinolysis
massive axonal demylination in pontine white matter 2ry to rapid osmotic changes ,
most commonly iatrogenic correction of hyponatremia
acute paralysis dysarthria ,dysphagia ,diplopia ,loss of consciousness
can caused locked in syndrome
correction
from low to high your pons will die
from high to low your brains will blow
acute inflammatory demylinenating poly neuropathy
most common gullien barre
depressed and absent dtr ,facial muscle palsy autonomic dysregulation
inc csf protein with normal cell count
iv immunoglobulins with no role for steroids
charcot marie tooth disease
hereditary motor and sensory neuropathy
autosomal dominant foot deformities
pmp22 genedup
pml
due to destruction of oligodendrocytes
severe immunosupression aids organ transplant
inc risk with natulizimab and rituximab
Krabbe
polyneuropathy destruction of oligodendrocytes ,developmental delay optic atrophy globoid cells
galactocerebrosidase, ar
metachromatic leukodystrophy
central and peripheral demyelination ,ataxia dementia arylsulfatase a cerebroside sulfate
neiman pick
progressive neurodegeneration ,hepatosplenomegaly ,foam cells ,cherry red spot on macula
sphingomyelinase
sphingomyelin
autosomal recessive
tay sachs
progressive neurodegeneration developmental delay hyperreflexia hyperacusis cherry red spot on macula lipid accumulation in ganglion cell layer ,lysosomes with onion skin no hepatosplenomegaly hexosaminidase A gm2 ganglioside AR
adrenoleukodystrophy
autosomal recessive disorder of peroxisome biogenesis due to mutated pex genes
hypotonia seizures hepatomegaly early death
struge weber syndrome
congenital non heriditary anomaly of neural crest derivatives
somatic mosaicism activating mutation in one copy of the GNAQ gene
capillary vascular malformation
port wine stain
cn 51 52
ipsilateral leptomeningeal angioma with calcification
seizures with eplipsy
struge weber syndrome
congenital non heriditary anomaly of neural crest derivatives
somatic mosaicism activating mutation in one copy of the GNAQ gene
capillary vascular malformation
port wine stain
cn 51 52
ipsilateral leptomeningeal angioma with calcification
seizures with eplipsy
struge weber syndrome
congenital non heriditary anomaly of neural crest derivatives
somatic mosaicism activating mutation in one copy of the GNAQ gene
capillary vascular malformation
port wine stain
cn 51 52
ipsilateral leptomeningeal angioma with calcification
seizures with eplipsy
struge weber syndrome
congenital non heriditary anomaly of neural crest derivatives
somatic mosaicism activating mutation in one copy of the GNAQ gene
capillary vascular malformation
port wine stain
cn 51 52
ipsilateral leptomeningeal angioma with calcification
seizures with eplipsy
intellectual disability
episcleral hemangioma
inc iop early onset glaucoma
also called encephalotrigeminal angiomatosis
tuberous sclerosis
ad variable expression mut in tsc1 on chromosome 9 , TSC2 on chromosome 16 , hemartomas in cns and skin ,angiofibromas ,mitral regurgitation ,ash leaf spots ,cardiac rhabdomyoma ,intellectual disability renal angiomyolipoma seizures ,shagreen patches autosomal dominant inc incidence of subependymal gaint cell astrocytomas ungal fibromas
neurofibromatosis type 1
autosomal dominant
100% penetrance
mutation in NF1 tumor suppressor gene
neurofibromatosis type 1
autosomal dominant 100% penetrance mutation in NF1 tumor suppressor gene on chromosome 17 (17 letters in von recklinghausen ),encodes neurofibromin a negative ras regulator cafe au lait spots intellectual disability cutaneous neurofibromas lisch nodules optic gliomas pheochromocytoma seizures signs of meningioma bone lesions eg sphenoid dysplasia CICLOPSS
neurofibromatosis type 2
ad mutation in NF2 tumor supressor gene on chromosome 22
bilateral vestibular schwannomas juvenile cataracts meningomas ,ependymomas
von hippel lindau disease
autosomal dominant
deletion in vhl gene on chromosome 3p
ubiquinates hypoxia inducible factor 1a
hemangioblastomas (high vascularity with hyperchromatic nuclei )in retina ,brain stem ,cerebellum , spine ,angiomatosis ,bilateral renal cell ca ,pheochromocytomas
pilocytic astrocytoma
most common brain tumor in childhood well circumscribed most often found in post fossa maybe supratentorial good prognosis astrocyte origin gfap bipolar neoplastic cells with hair like projections ass with microcysts and rosenthal fibers (eosinophilic corkscrew fibers cystic solid)
medulloblastoma
most common malignant brain tumor in childhood
cerebellum commonly
can compress 4thv non communicating hydrocephalus
headaches papilledema
can involve cerebellar vermis ,truncal ataxia
drop mets to spinal cord
form of primitive neuroectodermal tumor
homer wright rosettes small blue cells
synaptophysin +
ependymoma
most commonly found in 4th ventricle can cause hydrocephalus poor prognosis ependymal cell origin characteristic perivascular psuedorosettes .rod shaped blepharoplasts basal ciliary bodies found near the nucleus
craniopharyngioma
most common childhood supratentorial tumor ass with high recurrence rate
derived from rathkes pouch
cholesterol crystals fount in motor oil like fluid within tumor
pinealoma
can cause parinaud ,obstructive hydrocephalus, precocious puberrty in males
