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NMSP > neoplasms and related lesions of the nervous system > Flashcards

neoplasms and related lesions of the nervous system Flashcards

1
Q

70% of intracranial tumors arise in the _ in kids

in the _ in adults

A

posterior fossa in kids

in cerebral hemisphere in adults (above the tentorium)

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2
Q

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

A

location

infitrative

metastasize

CSF

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3
Q

most common place for pediatric brain tumors?

adults?

A

children: cerebellum and brainstem

adults: frontal, temporal, parital, meninges

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4
Q

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 _

A

children

supretentorial

infratentorial

adults, seziure

children

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5
Q

what are the common pediatric brain tumors

A

NEE-CAN

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

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6
Q

grading of brain tumors

Grade 1-

Grade 2-

Grade 3-

Grade 4-

A

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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7
Q

what are the 4 histological parameters for a glioma

A

nuclear atypia

mitoses

microvascular proliferation

necrosis

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8
Q

grading of a gliosis

A

Grade II: one parameter (atypia)

Grade III: two parameters (anaplasia, mitosis)

Grade IV: 3 or 4 parameters

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9
Q

grade I/IV astrocytoma is considered a _ astrocytoma

A

pliocytic

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10
Q

grade II/IV astrocytoma is considered a _ astrocytoma

A

diffuse

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11
Q

grade III/IV astrocytoma is considered a _ astrocytoma

A

anaplastic

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12
Q

a grade IV/IV astrocytoma is considerd a ?

A

glioblastoma/GBM

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13
Q

and oligodenfroglioma is what stage

A

II/IV or III/IV

2 or 3

  • has a better survivial rate
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14
Q

what are the types of gliomas

A

Astrocytomas
oligodendrogliomas
epdenymomas

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15
Q

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

A

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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16
Q

astrocytomas are usually _ matter lesions

A

white

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17
Q

brainstem astrocytoms have CN _ dysfunction

A

VI (6)

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18
Q

infiltrating astrocytomas are grades _ to _

they account for 80% of primary adult brain tumors

symptoms:

Better prognosis vs poor prognosis

A

II - IV (gliobastoma)

Seizures, Headaches, Focal Neuro Defecits

IDH mutatant- better
IDH wild type- poor

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19
Q

gross apperance of an infiltrating astrocytoma

A

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

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20
Q

histology of a grade II-IV astrocytoma

staining:

grade II = diffuse

A

hypercellular, elongated hyprechromatic nuclei

GFAP+ - fibrially background

IDH1 R132H immunohistochemical stains

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21
Q

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

A

cerebral hemisphere/cortex

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22
Q

diffuse astrocytoma can also show _ satellitosis in histology

A

perineuronal

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23
Q

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 _ _

A

2 decades of life (children)

cerebellum

IAA1549- Braf

MAP kinase pathway

NF1; neurofibromin

cystic with a mural nodule

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24
Q

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

A

biphasic

glial and cystic

hair-like

bipolar

rosenthal fibers

granular bodies (EGBs)

subarachnoid space

GFAP+

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25
Q

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

A

IV

throughout

CDKN2A

EGFR and PDGFR

TP53

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26
Q

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 _

A

older

wild

7

10

TERT and EGFR

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27
Q

secondary glioblastomas are in _ pts and is preceded by _

it has a IDH _ type and has a better prgnosis than _ and IDH2

A

younger

lower grade lesion

IDH1

IDH wild type

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28
Q

in glioblastomas there is a contrast _ enhancing lesion and central _

cells in a GBM follow _ tracts and extend well beyond imaging

A

ring

fiber

29
Q

histology of a GBM (3 things)

A

necrosis with a serpentine pattern

pseudopallisading

vascular/endothelial proliferation

30
Q

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.

A

VEGF

hypoxia

glomeruloid

31
Q

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)

A

4-5th decade

cerebral hemispheres

white

neurologic complaint for SEVERAL years (indolent/slow growing)

calcifcations

perineuronal satellitosis; perivascualr aggregation, supial

halos

anastamosing (chicken wire)

32
Q

most oligodendrocytes are _ differentiated grade _/IV and has the _ prognosis among all glial tumors

_ and _ have a favorable prognosis

_ is also a favorable prognosis

A

well

II/IV

best

IHD2 and IDH1

1p19Q

33
Q

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 _

A

III/IV

vascular

glioblastoma

34
Q

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

grade _/IV

A

first 2 decades

fourth ventricle

spine for adults (NF2)

grade II/IV

35
Q

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

A

exophytic (enhancing as well)

36
Q

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

A

cystic and paraventricular

37
Q

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

A

supratentorial

38
Q

what is seen in ithe histology of an ependymoma

A

round nuceli

fibrillay cytoplasmic processes (fibrillary background)

ependymal rosettes

39
Q

_ rosettes are more diagnostic than _ rosettes

A

true

perivascular

40
Q

what are true rosettes

A

there is a tubular structure with a central opening

41
Q

what is a perivascular pseudorosette

A

thin ependymal processes radiating toward the central blood vessel

more common of the two ( blood vessel in center)

42
Q

homer-wright rosettes are in what procesess

A

medullobasltoma and neuroblastoma

have a neuropill in the center

43
Q

flexner/true rosettes are in what processes

A

ependymoma and retinoblastoma

lumen in the center

44
Q

pseudo rosettes are in what procesess

A

ependymoma

vessel in center

45
Q

mxyopapillary epndeymoma is grade _/IV and presents with _ _

there are _ tumor cells arranged in a papillary patter around a _fibrovascular core

A

I/IV

filum terminale

cuboidal

mucin-fibrovascular core

46
Q

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

A

I/IV

lateral or 4th ventricle

obstructive
hydrocephalus

ependymal

fibrillary

47
Q

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 _

A

children

lateral ventricles (4th ventricle in adults)

obstruction

CSF

48
Q

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

A

3rd

foramen of monro

noncommunicating

positional

49
Q

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

neuronal and glioneuronal tumors

A

I/IV

glial and neuronal

seizures

temporal

BRAF gene

50
Q

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

neuronal and glioneuronal tumors

A

I/IV

epilepsy

temporal lobes

mucin-rich (floating neurons)

oligodendrocyte like cells (arranged in columns)

51
Q

embryonal tumors are mostly _ tumors and apperar primitive and undifferentiated

_ is the most common

other types (2)

A

children

medulloblastoma

atypical teratoid/rhaboid tumor
primitive neuroectodermal tumor

52
Q

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

A

IV/IV

WNT, SHH, Group 2, Group 4

53
Q

WNT medulloblastoma tumors are in _ kids is associated with chromosome _ and _

it is usually located in the ?

A

older

6

B catenin

located in the anterior 4th ventricle/middle cerebral peduncle

54
Q

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

usually located in the _ or _ _

A

infants- young

MYCN

vermis or lateral cerebellum

55
Q

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

A

MYC

17(I17Q)

worst prognosis

56
Q

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

A

I17Q

no MYC

plus or minus MYCN

57
Q

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 _ _)

A

cerebllum

subarachnoid space

drop

cada equina

58
Q

histology of medulloblastoma

classic:

desmoplasitc:

large cell/anaplastic

A

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)

59
Q

medulloblastoma is extremely _

A

radiosensitive

60
Q

desmoplastic medulloblastoma has a _ gene association

A

PTCH1

better prognosis

61
Q

medulloblastoma with extensive nodularity is associatd with

A

MBEN

62
Q

molecular subtypes of medulloblastoma

A

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

63
Q

PTCH1 is associated with

A

nevoid basal cell carcinoma syndrome

64
Q

APC is part of _

A

WNT

65
Q

TP53 is a part of _

A

SHH

66
Q

atypical teratoid/rhaboid tumor is a grade _/IV is typically located in the _ fossa

they have a divergent differentiation with what components

A

IV

posterior

epithelial, mesenchymal, neuronal, glial

67
Q

markers for atypical teratoid/rhaboid tumor

A

EMA and vimentin

68
Q

what cells are in atypical/rhaboid tumor

A

rhabdoid cells (eosinophilic cytoplasm and eccentric nucleus)

69
Q

Atypical teratoid/rhaboid tumor : 90% have chromosome _ with hSNF5/INI1

highly aggressive if less than 3 yo

A

22

NMSP (13 decks)

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