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BMB Test 3 > B7-058 Brain Tumors 2 > Flashcards

B7-058 Brain Tumors 2 Flashcards

1
Q

adult-type diffuse gliomas [3]

A

astrocytoma
oilgodendroglioma
glioblastoma

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2
Q

you can only grade CNS tumors using

and you should write out what when assigning grade?

A

arabic numerals 1, 2, 3, 4

“CNS WHO Grade”

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3
Q

most important first step in grading gliomas is determining […] type

A

IDH

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4
Q

lower grade and better prognosis gliomas have […]

A

IDH mutations

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5
Q

vast majority of IDH mutations in gliomas will have a point mutation at

A

R132H

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6
Q

gene encoding cytosolic nicotinamide adenine dinucleotide phosphate-dependent isocitrate dehydrogenase

A

IDH

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7
Q

IDH mutation in diffuse glioma is associated with [better/worse] prognosis

A

better

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8
Q

IDH mutations in diffuse gliomas are associated with […] age of onset

A

younger

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9
Q

how do we test for IDH mutations?

A

immunostain for IDH1-R132H

(most common mutation)

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10
Q

If immunostain for IDH1-R132H is negative but diffuse glioma is strongly suspected, what is the next best test?

A

Next Generation Sequencing (NGS)

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11
Q

discovers “non-canonical” IDH mutations

A

Next Generation Sequencing (NGS)

(mutations other than R132H)

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12
Q

IDH immunostain takes […] days, NGS takes […] days

A

1-2
10-14

(cannot continue work up until IDH status is known)

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13
Q

high incidence in diffuse astrocytomas

lower incidence in oligodendrogliomas, oligoastrocytomas, and glioblastomas

A

ATRX mutation

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14
Q

[…] expression is mutually exclusive with 1p/19p co-deletions

A

ATRX

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15
Q

ATRX expression is mutually exclusive with […] co-deletions

A

1p/19p

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16
Q

if you have a […] mutation you are very likely to have a diffuse astrocytoma

if you do not, you are very unlikely to have diffuse astrocytoma

A

ATXR

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17
Q

when IDH mutation is present, the immunostain is […]

A

positive (tissue turns brown)

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18
Q

when ATRX mutation is present, the immunostain is […]

A

negative (loose stain)

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19
Q

interpret the result of this ATRX immunostain

A

not mutated

retained expression

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20
Q

interpret the result of this ATRX immunostain

A

mutated

loss of expression
very likely diffuse astrocytoma

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21
Q

ATRX mutations are associated with IDH mutations and […] mutations

A

p53

(usually tested for with p53 immunostain, but more conclusively with NGS)

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22
Q

IDH mutant
tp53 mutant
ATRX mutant

molecular signature of […]

A

diffuse astrocytoma

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23
Q

poor prognostic factor in IDH mutant astrocytomas

A

homozygous deletion of CDKN2A/B

(automatically makes it CNS WHO grade 4)

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24
Q

presence of […] mutation automatically makes a IDH-mutant astrocytoma CNS WHO grade 4

A

homozygous deletion of CDKN2A/B

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25
Q

how to test for deletion of CDKN2A/B? [3]

A

NGS
FISH
CMA (what KU uses)

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26
Q

a technique used to detect the presence or absence of specific gene sequences

A

FISH

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27
Q

used to detect chromosomal alterations

A

FISH

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28
Q

very expensive, very comprehensive test used to analyze genomic copy number variation that can detect very small deletions and duplications

A

CMA

(basically the entire genome)

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29
Q

turn around time for CMA

A

14 days

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30
Q

essential for both grade and prognosis of diffuse astrocytoma

A

CMA for CDKN2A/B

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31
Q

what mutations are required for diagnosis of oligodendrogliomas? [2]

A

IDH mutations
1p/19p co-deletions

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32
Q

tests for 1p/19p co-deletions [2]

A

FISH
CMA if FISH doesnt pick it up

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33
Q

IDH mutation
1p/19p co-deletions

molecular signature of […]

A

oligodendroglioma

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34
Q

molecular signatures of IDH wildtype gliomas [3]

A

EGFR amplification
whole chromosome 7 gain and 10 loss (+7/-10)
TERT promoter mutation

(only need 1 to classify as WHO grade 4)

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35
Q

[…] confers a better prognosis for IDH wildtype gliomas

A

MGMT methylation

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36
Q

MGMT is associated with long survival in patients treated with […]

A

alkylating agents (temozolomide)

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37
Q

why is MGMT methylation considered a good prognostic indicator for IDH wildtype gliomas?

A

silences the gene so there is no DNA repair
so, the tumor cells are more sensitive to DNA damage from alkylating agents

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38
Q

how do you test for MGMT methylation?

A

PCR analysis

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39
Q

are CNS gliomas staged?

A

no

grade

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40
Q

first step in molecular classification of gliomas

A

IDH status

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41
Q

common, highly malignant primary brain tumor with less than one year median survival

A

glioblastoma

42
Q

grade 4 astrocytoma is called

A

glioblastoma

43
Q

“butterfly” glioma

A

glioblastoma

(can cross corpus callosum resulting in this shape)

44
Q

glioblastoma is associated with […] amplification

45
Q

astrocyte origin
GFAP+

A

glioblastoma

46
Q

pseudo-palisading pleomorphic tumor cells
central necrosis
microvascular proliferation

A

glioblastoma

47
Q

relatively rare, slow growing primary brain tumor
often calcified

A

oligodrendroglioma

48
Q

“fried egg” cells (round nuclei with clear cytoplasm)

“chicken wire” capillary pattern

A

oligodrendroglioma

49
Q

ring enhancing lesion with edema
butterfly lesion crossing the midling

A

glioblastoma

50
Q

criteria to make a histologic diagnosis a high grade glioma or glioblastoma

A

3 of the following:
Atypia
Mitoses
Endothelial proliferation
Necrosis

AMEN mneumonic

51
Q

hypercellularity
dense cells

A

example of atypia

52
Q

what is circled?

A

mitotic figures

53
Q

what are these?

A

vessels (endothelial proliferation)

54
Q

what is this showing?

A

pseudo palisading necrosis

(viable cells on edge)

55
Q

in patients above […] the likelihood of finding an IDH mutation that is not IDH1-R132 drops significantly

56
Q

what type of testing is typically done for young patients with high grade gliomas without IDH mutation?

A

NGS

(under 55ish)

57
Q

what is the prognostic marker for high grade IDH wildtype gliomas?

A

MGMT

methylation = good
no methylation= bad

58
Q

what grades of IDH mutant astrocytomas are possible?

59
Q

mitoses in astrocytoma automatically makes it grade

60
Q

astrocytoma with just atypia and negative FISH and CMA is grade

61
Q

astrocytoma with atypia mitoses, necrosis and/or MVP

62
Q

what grades can oligodendroglioma be

63
Q

calcifications
perinuclear haloes “fried egg”
chicken wire vasculature

A

oligodendroglioma

64
Q

artefact of fixation, only seen on FFPE tissue

A

perinuclear haloes “fried egg”

oligodendroglioma

65
Q

first thing you do on any diffuse glioma

66
Q

oligodendroglioma with no mitoses, no MVP, no necrosis is grade

67
Q

oligodendroglioma with mitoses OR MVP OR necrosis is grade

68
Q

infiltrative mid-line high grade glioma with predominantly astrocytic differentiation

A

diffuse midline glioma H3 K27 altered

69
Q

must have K27M mutation or alteration in H3 K27

A

diffuse midline glioma H3 K27 altered

70
Q

diffuse midline glioma H3 K27 altered is grade

A

4

automatically

71
Q

H3 K27M immunostain positive

A

diffuse midline glioma H3 K27 altered

72
Q

rosettes or pseudorosettes
GFAP+

A

ependymoma

73
Q
A

myxopapillary ependymoma

74
Q

ependymoma occurring in the extramedullary sacral area

A

myxopapillary ependymoma

75
Q

by definition, myxopapillary ependymoma are grade

A

2 (no grade 1)

76
Q

circumscribed tumor arising in cerebellum
occurs in children

A

pilocytic astrocytoma

77
Q

arises in cerebellum and cerebral midline, optic pathways, hypothalamus, and brainstem

A

pilocytic astrocytoma

78
Q

cyst with mural nodule
biphasic compact bipolar cells
looser multipolar cells with microcysts

A

pilocytic astrocytoma

79
Q

BRAF-KIAA1549

A

pilocytic astrocytoma

80
Q

patients with NF1 generally have […] pilocytic astrocytomas

A

optic pathway

81
Q

by definition, pilocytic astrocytoma is grade

82
Q

high grade small round blue cell tumors
presenting mainly in childhood

A

medulloblastoma

83
Q

medulloblastoma is grade

A

4
automatically

84
Q

an embryonal neuroepithelial tumor arising in the cerebellum or dorsal brain stem

A

medulloblastoma

85
Q

most common primary brain tumor in childhood

A

pilocytic astrocytoma

86
Q

often found in the posterior fossa of children

A

pilocytic astrocytoma

87
Q

often found in the posterior fossa of adults

A

hemangioblastoma

88
Q

bipolar neoplastic cells with hairlike projections
microcysts
rosenthal fibers

A

pilocytic astrocytoma

89
Q

most common malignant brain tumor in childhood

A

medulloblastoma

90
Q

can compress the 4th ventricle causing noncommunicating hydrocephalus, headaches, and papilledema

can involve the cerebellar vermis causing ataxia

A

medulloblastoma

91
Q

Homer-Wright rosettes

(small blue cells surround central area of neurophil)

A

medulloblastoma

92
Q

most commonly found in the 4th ventricle
can cause hydrocephalus

A

ependymoma

93
Q

characteristic perivascular pseudorosettes

rod-shaped blepharoplasts found near the nucleus

A

ependymoma

94
Q

diffuse midline glioma, H3 K27M altered is automatically grade

95
Q

defined molecularly by co-deletions of chromosomes 1p and 19q AND mutation in IDH1/2

A

oligodendrogliomas

96
Q

histologic finding associated with long standing gliosis, slow growing astrocytic tumors, and walled off infarcts

A

rosenthal fibers

97
Q

what is this?

A

pseudopalisading necrosis

98
Q

glioblastomas are grade […] astrocytomas

99
Q

enhancing on imaging is usually associated with […] grade gliomas

100
Q

partially solid and cystic tumor of the cerebellum that contains many rosenthal fibers
in children

A

pilocytic astrocytoma

101
Q

[…] are only seen in oligodendrogliomas and are an artefact of formalin fixation

A

halos

(do not see halos on frozen tissue or fresh)

BMB Test 3 (18 decks)

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