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67 Cards in this Set

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IDH1

Isocitrate dehydrogenase I (IDH 1) a metabolicenzyme in the citric acid cycle, is commonlymutated in astrocytomas, oligodendroglioma,and mixed gliomas- low grade (II) and anaplastic(III).

Is IDH 1 mutation found in primary or secondary GBM?

IDH1 and IDH2 are uncommon in primary GBMs(~5%) but can be seen in secondary GBMsarising from lower grade gliomas.

Is IDH 1 and 2 mutation good or bad prognosis?

good



MGMT

O-6-methylguanine-DNAmethyltransferase is a DNA repair enzy that removes ALKYL group


thought to predict response to alkylating agents

what happens if the MGMT gene is hypermethylated?

better prognosis

cell of origin of meningiomas

arachnoidal cells

what meningioma associated with NF2

SPORADIC MENINGIOMAS

WHO GRADE I MENINGEOMA?

fibrous


psammomatous


classic (meningothelial)


secretory


microcystic


lymphoplasmacytic


metaplastic


angiomatous

WHO GRADE II MENINGEOMA?

CLEAR CELL


CHORDOID

WHO GRADE III MENINGEOMA?

PAPILLARY


RHABDOID


ANAPLASTIC

MENINGIOMA STAINS?

EMA+


S100+


ck-


mib1 > 4% increase recurrence


cd34+ fibrous meningioma


gfap -


cea + secretory



what is the most common chromosomal abnormality?

CH 22

what us the recurrence rate for anaplastic MENINGIOMA?

50-90%

Cerebellar lesion with nidus

pilocytic astro


or


hemgioblastoma

Kid with medication resistant seizure?

DNT (dysemberyoplatic neuroeithelioma)

what is DNT associated with ? genetic disorder?

Gorlin syndrome

meduloblastoma commonest genetic alteration

Isochromosome 17

is isochromosome 17 of meduloblastoma a good or bad prognosis

bad

classic autopsy finding in methanol intoxication?

bilateral (putamen, GP necrosis)

when duo see nucleolin in Astrocytomas

gamistocytic variant

who grade 2 astrocytomas?

gamistocytic


granular cell


microcystic

who grade 3 astrocytomas?

anaplastic astrocytoma


gliomatosis cerebri

who grade 4 astrocytomas

GBM

anaplastic astrocytoma of children?

pontine location

in anaplastic astrocytoma what is the microscopic features?

Increased cellularity


Pleomorphism and hyperchromasia


Variable mitotic activity

Increased cellularity


Pleomorphism and hyperchromasia


Variable mitotic activity


are seen in?

anaplastic astrocytoma

microscopic features of GBM?

cytologicatypia and mitotic activity, as well as endothelialproliferation and/or necrosis

WHAT MOLECULAR MUTATION IN BRAIN STEM GBM OF KIDS

p53 gene and chromosome 17 p alterations without EGFR geneamplification

p53 gene and chromosome 17 p alterations without EGFR gene amplification IS SEEN IN?

BRAIN STEM GBM OF KIDS

MGMT ENZYME, WHAT HAPPENS IF THE GENE GETS METHYLATED?

MGMT PROTEIN GETS REDUCED AND SO INCREASE RESPONSE TO TEMOZOLIMIDE TREATMENT

P53 17P ALTERATION IS FOUND ALSO IN?

59% ASTROCYTOMAS


65% SECONDARY GBM


84% giant cell gbm


26% gliosarcoma


28% primary gbm

small cell GBM

Primary GBM


More common in elderly


EGFR overexpression


Dismal prognosis


Mimic oligodendrglioma

gliosarcoma who grade?

who grade 4

gliosarcoma histology

Biphasic tissue pattern


- GFAP positive astrocytic and


- GFAP negative mesenchymalcomponents


- Reticulin rich


- Bone, cartilage and striated muscle differentiation

NF1 gene maps to 17q11.2

pilocytic astrocytoma

pilocytic astrocytoma microscopic pattern

bipolar fibrillated astrocytes


rosenthal fibers


EGB

cerebellar cyst with mural nodule?

Pilocytic astrocytoma

childhood astrocytoma

pilomyxoid astrocytoma

location of pilomyxoid astro?

Hypothalamic/chiasmal region

superficial partially leptomeningeal cyst with mural nodule?

pleomorphic xanthoastro (PXA)

location of PXA

SUPRATENTORIAL USALLY TEMPORAL

EGB ARE SEEN IN?

PILOCYTIC ASTRO


PILOMYXOID ASTRP


PXA

RESTICULIN STAIN IN PXA

POSITIVE

location of SEGA

WALL OF LATERAL VENT

ASSOCIATION WITH SEGA

TUBEROUS SCLEROSIS

SEGA

- Large cells with abundant, “glassy” cytoplasm to smaller, spindled forms


– Largeneuron-like cells with prominent nucleoli

EPIDURAL HEMATOMA

MIDDLE MENINGEAL ARTERY

SUBDURAL

BRIDGING VEINS

subfacial (cingulate) herniation

due to mass effect the cingulate gyrus herniates under the falx cerebri


compression of anterior cerebral artery

transtintorial herniation

Anteromedial aspect of parahippocampal gyrus underfree edge of tentorium


compreesion of posterior cerebral artery and third crainal n

tonsillar herniation

Downward displacement ofcerebellar tonsils into foramenmagnum


compression of medulla

lewy bodies

dense cytoplasmic inclusion with peri-inclusionhalo


parkinson disease and aging

staining for Lewy bodies

Ubiquitin (+)


α-synuclein (+)


Tau protein (−)


Actin (−)

neurofibrally tangles

Flame shaped• Pyramidal neurons


AD, NORMAL AGING

staining for tangles

Ubiquitin (+/-)


Tau protein (+)

Granulovacular degeneration?

Small vacuoles containingsmall, dense, round,basophilic granules


present in AD


PICK


Normal aging




present in CA1 region of hippo



staining for vascular degeneration

Ubiquitin (+)


Tau protein (+)

hirano bodies

Eosinophilic rod-shaped,refractile inclusions


present in AD


PICK


Normal aging




present in CA1 region of hippo

staining for Hirano bodies?

α – Actinin, Actin, Vinculin, Tropomyosin, Tau (+)

Bunina bodies

Small bead – like eosinophilicinclusions



Found in motor neuron disease (ALS)

Dystrophic neurites

Abnormally dilated neuronal processes


– Pass through amyloid plaques


• Alzheimer’s disease

ASTROCYTIC INCLUSIONS:

ROSENTHAL FIBERS


EGB


CORPORA AMYLACEA

ROSENTHAL FIBERS POSITIVE FOR?

GFAP,αB crystallin and ubiquitin

EGB POSITIVE FOR?

contain α–1– antitrypsin and chymotrypsin




Associated with low grade glial tr

corpora amylacea

Composed of glycogen-likecarbohydrates and proteins




aging

function of oligodendrocytes?

produce myelin for the CNS

where is the inclusions for PML found?

oligodendroglia


Progressive multifocal leukoencephalopathy Homogeneous, eosinophilic inclusion JC virus