neoplasms and related lesions of the nervous system Flashcards by Deja Gause

70% of intracranial tumors arise in the _ in kids

in the _ in adults

posterior fossa in kids

in cerebral hemisphere in adults (above the tentorium)

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benign intracranial tumors can be lethal due to _

_ pattern of growth makes intracranial tumors unresectable

tumors in the CNS rarely _

some pediatric tumors spread through the _ in the subarachnoid space

location

infitrative

metastasize

CSF

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most common place for pediatric brain tumors?

adults?

children: cerebellum and brainstem

adults: frontal, temporal, parital, meninges

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brain tumors are the second most common malignancy among _

location for adults:

location for kids:

Glioblastomas are common in _ with a new onset of _ think brain tumor!

Embryonal tumors and medulloblastomas are common in _

children

supretentorial

infratentorial

adults, seziure

children

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what are the common pediatric brain tumors

NEE-CAN

neuronal/glial, non-neuroectodermal, embryonal. ependymoma, chorid plexus, astrocytoma

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grading of brain tumors

Grade 1-

Grade 2-

Grade 3-

Grade 4-

grade 1: low proliferative potential (can resect)

grade 2- infiltrative, likely to recur, atypia

grade 3- anaplasia and mitosis need radiation/chemo

grade 4- fatal outcome; microvascular proliferation and necrosis

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what are the 4 histological parameters for a glioma

nuclear atypia

mitoses

microvascular proliferation

necrosis

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grading of a gliosis

Grade II: one parameter (atypia)

Grade III: two parameters (anaplasia, mitosis)

Grade IV: 3 or 4 parameters

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grade I/IV astrocytoma is considered a _ astrocytoma

pliocytic

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grade II/IV astrocytoma is considered a _ astrocytoma

diffuse

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grade III/IV astrocytoma is considered a _ astrocytoma

anaplastic

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a grade IV/IV astrocytoma is considerd a ?

glioblastoma/GBM

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and oligodenfroglioma is what stage

II/IV or III/IV

2 or 3

has a better survivial rate

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what are the types of gliomas

Astrocytomas
oligodendrogliomas
epdenymomas

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Grade 1 astrocytoma is usually in the _ decase

Grade II astrocytoma is usually in the _ decade

grade III astrocytome is usually in the _ decade

grade IV astrocytoma is also known as a _ and is usually seen in the _ decade

first

3-4 decade (1 bad feature)

5th decade (2 bad features)

glioblastoma; 6th decade and beyond (3 or 4 bad features)

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astrocytomas are usually _ matter lesions

white

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brainstem astrocytoms have CN _ dysfunction

VI (6)

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infiltrating astrocytomas are grades _ to _

they account for 80% of primary adult brain tumors

symptoms:

Better prognosis vs poor prognosis

II - IV (gliobastoma)

Seizures, Headaches, Focal Neuro Defecits

IDH mutatant- better
IDH wild type- poor

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gross apperance of an infiltrating astrocytoma

poorly defined, infiltrative, can be firm or gelatinous with cysts

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histology of a grade II-IV astrocytoma

staining:

grade II = diffuse

hypercellular, elongated hyprechromatic nuclei

GFAP+ - fibrially background

IDH1 R132H immunohistochemical stains

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diffuse astrocytomes (grade II - IV) are typically located in the _

cerebral hemisphere/cortex

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diffuse astrocytoma can also show _ satellitosis in histology

perineuronal

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Pliocytic astrocytomas (grade I/IV) are in the _ of life

located in the _. mostly

they have a _ gene mutation and genetic alterations in _ _ pathway

_ predisposes children to pliocytic astrocytomas especially optic nerve tumors (familial) there is a functional loss of _ in the tumor

apperance: well circumscribed and _ with a _ _

2 decades of life (children)

cerebellum

IAA1549- Braf

MAP kinase pathway

NF1; neurofibromin

cystic with a mural nodule

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pliocytic astrocytomas have a _ pattern with loose _ and _ changes and dense/compact piloid tissue

there are _ like cells with long _ processes

Long standing gliosis is common and _ fibers appear

there are eosiophilic _ _ as well

extension into the _ space is common

it will be _ positive (fibrillary meshwork)

pliocytic astrocytoma

biphasic

glial and cystic

hair-like

bipolar

rosenthal fibers

granular bodies (EGBs)

subarachnoid space

GFAP+

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Gliobastomas are _/IV astrocytomas that is the most common primary brain neoplasm distributed _ the brian the genetic abberation that allows it to escape normal growth controls is _ the activation of growth factor signaling pathways is regulated by _ and _ resistance to apoptosis is through _ mutations

IV throughout CDKN2A EGFR and PDGFR TP53

primary glioblastomas are in _ pt. with no precursor lesion they have the IDH _ type and a gain in chromosome _ and a loss of chromosome _ and mutation in _ and _

older wild 7 10 TERT and EGFR

secondary glioblastomas are in _ pts and is preceded by _ it has a IDH _ type and has a better prgnosis than _ and IDH2

younger lower grade lesion IDH1 IDH wild type

in glioblastomas there is a contrast _ enhancing lesion and central _ cells in a GBM follow _ tracts and extend well beyond imaging

ring fiber

histology of a GBM (3 things)

necrosis with a serpentine pattern pseudopallisading vascular/endothelial proliferation

vascular and endothelial proliferation in a GB is due to production of _ by malignant astrocytes in response to _ . Tufts of cells pile up and bulge into the vascular lumen, a _ body forms.

VEGF hypoxia glomeruloid

Oligodenrogliomas are usually found in the _ decade and are primarly in the _ _ with a predilection for _ matter. symptoms _ are restricted to the cortex or have a gyriform distribution histology: _ satellitosis, _ aggregation and _ accumulation of cells, there will be perinuclear _ and delicate _ of capillaries (known as chicken wire)

4-5th decade cerebral hemispheres white neurologic complaint for SEVERAL years (indolent/slow growing) calcifcations perineuronal satellitosis; perivascualr aggregation, supial halos anastamosing (chicken wire)

most oligodendrocytes are _ differentiated grade _/IV and has the _ prognosis among all glial tumors _ and _ have a favorable prognosis _ is also a favorable prognosis

well II/IV best IHD2 and IDH1 1p19Q

anaplastic oligodendrocytes are _/IV and have _ hypertrophy and necrosis there is an increase in nucleus to cytoplasmic ration, increase in mitosis, and increase in cellularity these have poor prognosis similar to a _

III/IV vascular glioblastoma

epdendymomas are usually in the _ decade of life and the most common site is ? and _ for adults grade _/IV

first 2 decades fourth ventricle spine for adults (NF2) grade II/IV

the growth of an ependymoma is _ if it grows on the 4th ventricle

exophytic (enhancing as well)

the growth of a ependymoma if it is in the supratentorial region is?

cystic and paraventricular

if an ependymoma is grade III it is usually _ with mitosis, has microvascular proliferation, necrosis

supratentorial

what is seen in ithe histology of an ependymoma

round nuceli fibrillay cytoplasmic processes (fibrillary background) ependymal rosettes

_ rosettes are more diagnostic than _ rosettes

true perivascular

what are true rosettes

there is a tubular structure with a central opening

what is a perivascular pseudorosette

thin ependymal processes radiating toward the central blood vessel ## Footnote more common of the two ( blood vessel in center)

homer-wright rosettes are in what procesess

medullobasltoma and neuroblastoma have a neuropill in the center

flexner/true rosettes are in what processes

ependymoma and retinoblastoma lumen in the center

pseudo rosettes are in what procesess

ependymoma vessel in center

mxyopapillary epndeymoma is grade _/IV and presents with _ _ there are _ tumor cells arranged in a papillary patter around a _fibrovascular core

I/IV filum terminale cuboidal mucin-fibrovascular core

subeoendymoma is a grade _/IV and is in the _ or _ ventricle under the epdenymal layers, it is slow growing and is solid. It is usually an incidential finding and can cause _ _ histology: cluster of _ nuclei in a _ background

I/IV lateral or 4th ventricle obstructive hydrocephalus ependymal fibrillary

choroid plexus tumors: papilloma most common in _ and in the _ _ Hydrocephalus due to _ is most common a choid plexus carcinoma has a increased production of _

children lateral ventricles (4th ventricle in adults) obstruction CSF

colloid cysts (choriod pelxus tumor) is in the _ ventricle and is toung adults it obstructs the _ _ _ and creates a _ hydrocephalus it can be _ and can be rapidly fatal

3rd foramen of monro noncommunicating positional

ganglioglioma is grade _/IV and it is a mix of mature _ and _ cells these are superifical lesions that present as _ that are refractory to treatment they are usually in the _ lobes and grow slowly activating mutation ## Footnote neuronal and glioneuronal tumors

I/IV glial and neuronal seizures temporal BRAF gene

Dysembryoplastic neuroepithelial tumor (DNET) is a grade _/IV and is associated with _ it is located in the _ lobes and has multiple _ rich intracortical nodules of small _ like cells ## Footnote neuronal and glioneuronal tumors

I/IV epilepsy temporal lobes mucin-rich (floating neurons) oligodendrocyte like cells (arranged in columns)

embryonal tumors are mostly _ tumors and apperar primitive and undifferentiated _ is the most common other types (2)

children medulloblastoma atypical teratoid/rhaboid tumor primitive neuroectodermal tumor

medulloblastoma is a grade _/IV what are the 4 molecular groups

IV/IV WNT, SHH, Group 2, Group 4

WNT medulloblastoma tumors are in _ kids is associated with chromosome _ and _ it is usually located in the ?

older 6 B catenin located in the anterior 4th ventricle/middle cerebral peduncle

SHH medulloblastoma is common in _ and a _ amplification the prognosis is in between WNT and groups 3 and 4 usually located in the _ or _ _

infants- young MYCN vermis or lateral cerebellum

group 3 medulloblastoma has a _ amplification and is on what chromosome. It has the _ prognosis

MYC 17(I17Q) worst prognosis

group 4 medulloblastomas is associated with _ with no _ , +/- _

I17Q no MYC plus or minus MYCN

medulloblatoma is usually in the _ and midline and occludes CSF flow it has a propensity ro spread into the _ space, there are _ metz (disseminate thru CSF to _ _)

cerebllum subarachnoid space drop cada equina

histology of medulloblastoma classic: desmoplasitc: large cell/anaplastic

sheets of anaplastic cells mitosis K1-67 is high classic: homer wright rosettes (synatrophysin) desmoplastic/nodular: arease of stroma in the nodule, collagen and reticulin with pale islands large/anaplastic: cell wrapping (cell in cell)

medulloblastoma is extremely _

radiosensitive

desmoplastic medulloblastoma has a _ gene association

PTCH1 ## Footnote better prognosis

medulloblastoma with extensive nodularity is associatd with

MBEN

molecular subtypes of medulloblastoma

WNT, SHH, Group 3 (WNT), group 4 (MYCN, CDK6)

PTCH1 is associated with

nevoid basal cell carcinoma syndrome

APC is part of _

WNT

TP53 is a part of _

SHH

atypical teratoid/rhaboid tumor is a grade _/IV is typically located in the _ fossa they have a divergent differentiation with what components

IV posterior epithelial, mesenchymal, neuronal, glial

markers for atypical teratoid/rhaboid tumor

EMA and vimentin

what cells are in atypical/rhaboid tumor

rhabdoid cells (eosinophilic cytoplasm and eccentric nucleus)

Atypical teratoid/rhaboid tumor : 90% have chromosome _ with hSNF5/INI1 highly aggressive if less than 3 yo