brainonco

Question 1

glioblastoma

Answer 1

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

Question 2

oligodendroglioma

Answer 2

relatively rare slow growing most often on frontal lobes
oligodendrocyte origin
fried egg cells round nuceli with clear cytoplasm
chicken wire capillary pattern

Question 3

meningioma

Answer 3

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

Question 4

hemangioblastoma

Answer 4

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

Question 5

pit adenoma

Answer 5

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

Question 6

schwannoma

Answer 6

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

Question 7

osmotic demyelination syndrome

Answer 7

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

Question 8

acute inflammatory demylinenating poly neuropathy

Answer 8

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

Question 9

charcot marie tooth disease

Answer 9

hereditary motor and sensory neuropathy
autosomal dominant foot deformities
pmp22 genedup

Question 10

pml

Answer 10

due to destruction of oligodendrocytes
severe immunosupression aids organ transplant
inc risk with natulizimab and rituximab

Question 11

Krabbe

Answer 11

polyneuropathy destruction of oligodendrocytes ,developmental delay optic atrophy globoid cells
galactocerebrosidase, ar

Question 12

metachromatic leukodystrophy

Answer 12

central and peripheral demyelination ,ataxia dementia arylsulfatase a cerebroside sulfate

Question 13

neiman pick

Answer 13

progressive neurodegeneration ,hepatosplenomegaly ,foam cells ,cherry red spot on macula
sphingomyelinase
sphingomyelin
autosomal recessive

Question 14

tay sachs

Answer 14

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

Question 15

adrenoleukodystrophy

Answer 15

autosomal recessive disorder of peroxisome biogenesis due to mutated pex genes
hypotonia seizures hepatomegaly early death

Question 16

struge weber syndrome

Answer 16

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

Question 16

struge weber syndrome

Answer 16

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

Question 16

struge weber syndrome

Answer 16

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

Question 16

struge weber syndrome

Answer 16

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

Question 17

tuberous sclerosis

Answer 17

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

Question 18

neurofibromatosis type 1

Answer 18

autosomal dominant
100% penetrance
mutation in NF1 tumor suppressor gene

Question 19

neurofibromatosis type 1

Answer 19

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

Question 20

neurofibromatosis type 2

Answer 20

ad mutation in NF2 tumor supressor gene on chromosome 22

bilateral vestibular schwannomas juvenile cataracts meningomas ,ependymomas

Question 21

von hippel lindau disease

Answer 21

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

Question 22

pilocytic astrocytoma

Answer 22

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)

Question 23

medulloblastoma

Answer 23

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 +

Question 24

ependymoma

Answer 24

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

Question 25

craniopharyngioma

Answer 25

most common childhood supratentorial tumor ass with high recurrence rate
derived from rathkes pouch
cholesterol crystals fount in motor oil like fluid within tumor

Question 26

pinealoma

Answer 26

can cause parinaud ,obstructive hydrocephalus, precocious puberrty in males