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Tumour > Gliomas, glioneural and neuronal tumours_ CNS 5 WHO 2021 > Flashcards

Gliomas, glioneural and neuronal tumours_ CNS 5 WHO 2021 Flashcards

1
Q

What types of tumours are included in the tumour group “Adult-type diffuse gliomas”?

A
  • Astrocytoma, IDH-mutant
  • Oligodendroglioma, IDH-mutant and 1p/19q-codeleted
  • Glioblastoma, IDH-wildtype
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2
Q

What types of tumours are included in the tumour group “Paediatric-type diffuse low-grade gliomas”?

A
  • Diffuse astrocytoma, MYB- or MYBL1-altered
  • Angiocentric glioma
  • Polymorphous low-grade neuroepithelial tumour of the young
  • Diffuse low-grade glioma, MAPK pathway-altered
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3
Q

What types of tumours are included in the tumour group “Paediatric-type diffuse high-grade gliomas”?

A

Diffuse midline glioma, H3 K27-altered

  • Diffuse hemispheric glioma, H3 G34-mutant
  • Diffuse paediatric-type high-grade glioma, H3-wildtype and IDH-wildtype
  • Infant-type hemispheric glioma
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4
Q

What types of tumours are included in the tumour group “Circumscribed astrocytic gliomas”?

A
  • Pilocytic astrocytoma
  • High-grade astrocytoma with piloid features
  • Pleomorphic xanthoastrocytoma
  • Subependymal giant cell astrocytoma
  • Chordoid glioma
  • Astroblastoma, MN1-altered
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5
Q

What types of tumours are included in the tumour group “Glioneuronal and neuronal tumours”?

A
  • Ganglioglioma
  • Desmoplastic infantile ganglioglioma/desmoplastic infantile astrocytoma
  • Dysembryoplastic neuroepithelial tumour DNET
  • Diffuse glioneuronal tumour with oligodendroglioma-like features and nuclear clusters
  • Papillary glioneuronal tumour
  • Rosette-forming glioneuronal tumour
  • Myxoid glioneuronal tumour
  • Diffuse leptomeningeal glioneuronal tumour
  • Gangliocytoma
  • Multinodular and vacuolating neuronal tumour
  • Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease)
  • Central neurocytoma
  • Extraventricular neurocytoma
  • Cerebellar liponeurocytoma
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6
Q

What types of tumours are included in the tumour group “Ependymomas”?

A
  • Supratentorial ependymoma
  • Supratentorial ependymoma (ZFTA or YAP1 fusion-positive)
  • Posterior fossa ependymoma
  • Posterior fossa ependymoma (PFA or PFB group)
  • Pinal ependymoma
  • Spinal ependymoma, MYCN amplified
  • Myxopapillary ependymoma
  • Subependymoma
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7
Q

What does ATRX mutation stand for?

A

Alpha-thalassemia/mental retardation syndrome X

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8
Q

What does “CDKN2A/B” stand for?

A

Cyclin-dependent kinase inhibitor 2A/B

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9
Q

What does “EGFR” stand for?

A

Epidermal growth factor receptor

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10
Q

What does “H3 G34” stand for?

A

Histone H3 3 G34

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11
Q

What does “H3 K27M” stand for?

A

Histone H3 K27M

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12
Q

In what glioma type/s is “ZFTA fusions” clinically relevant?

A
  • Present in some supratentorial ependymomas (ZFTA: previously named RELA fusions)
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13
Q

What does “IDH1/2” mutation stand for?

A

Isocitrate dehydrogenase

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14
Q

What does “MAPK” stand for?

A

Mitogen-activated protein kinase pathway

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15
Q

What does “MGMT” stand for?

A

methylation O6-methylguanine DNA methyltransferase

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16
Q

In what glioma type/s is “Gain of chromosome 7/loss of chromosome 10 (+ 7/ − 10)” clinically relevant?

A
  • Common in glioblastoma IDH-wildtype CNS WHO grade 4
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17
Q

What does “TERTp” stand for?

A

Telomerase reverse transcriptase promotor

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18
Q

What does “YAP1 “ stand for?

A

Yes-associated protein 1

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19
Q

what does “ZFTA” stand for?

A

Zinc finger translocation associated

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20
Q

In what glioma type/s is “Loss of chromosome 1p and 19q (loss of heterozygosity) (1p/19q codeletion)” clinically relevant?

A
  • Prerequisite for the diagnosis of oligodendroglioma
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21
Q

In what glioma type/s is “H3 K27M mutation” clinically relevant?

A
  • One of the criteria of diffuse midline glioma, H3 K27M altered
  • May occur in other gliomas not located in the midline (pilocytic astrocytoma and ependymoma)
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22
Q

In what glioma type/s is “H3 G34 mutation

Histone H3 3 G34” clinically relevant?

A
  • Present in hemispheric diffuse glioma, IDH-wildtype, predominantly in children and young adults, poor prognosis
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23
Q

In what glioma type/s is “EGFR-mutations” clinically relevant?

A
  • Common in glioblastoma, IDH-wildtype CNS WHO grade 4
  • If present in astrocytoma, IDH-wildtype CNS WHO grades 2 or 3, it is consistent with glioblastoma, IDH-wildtype CNS WHO grade 4
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24
Q

In what glioma type/s is “EGFR-mutations” clinically relevant?

A
  • Most common is EGFRvIII, frequently present in glioblastoma, IDH-wildtype CNS WHO grade 4
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25
Q

In what glioma type/s is “H3 G34 mutation” clinically relevant?

A
  • Present in hemispheric diffuse glioma, IDH-wildtype, predominantly in children and young adults, poor prognosis
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26
Q

In what glioma type/s is “YAP1 fusions” clinically relevant?

A
  • Present in some supratentorial ependymomas, especially in paediatric tumours
27
Q

In what glioma type/s is “TP53 mutation” clinically relevant?

A
  • Present in most astrocytoma IDH-mutant, rare in oligodendrogliomas
28
Q

In what glioma type/s is “TERTp mutation” clinically relevant?

A
  • Present in most oligodendroglioma
  • If present in diffuse astrocytoma, IDH-wildtype CNS WHO grades 2 and 3 (i.e. without the histopathological hallmarks of glioblastoma (necrosis and/or microvascular proliferation)), it is consistent with glioblastoma IDH-wildtype CNS WHO grade 4
29
Q

In what glioma type/s is “MYB- or MYBL1-altered” clinically relevant?

A
  • Alterations typical for a paediatric low-grade glioma
30
Q

In what glioma type/s is “MGMT promotor methylation” clinically relevant?

A
  • DNA repair enzyme, methylation predicts good response to alkylating agents such as temozolomide in glioblastoma, IDH-wildtype
31
Q

In what glioma type/s is “MAPK” clinically relevant?

A
  • Alterations typical for paediatric-type diffuse low-grade gliomas
32
Q

In what glioma type/s is “KIAA1549-BRAF gene fusion” clinically relevant?

A

Frequently found in pilocytic astrocytoma, also in diffuse leptomeningeal glioneuronal tumour, pilomyxoid astrocytoma and gangliogliomae

33
Q

In what glioma type/s is “IDH1/2” clinically relevant?

A
  • Frequently mutated in diffuse gliomas (astrocytomas and oligodendrogliomas) and is associated with better prognosis than IDH-wildtype gliomas
34
Q

In what glioma type/s is “H3 K27M mutation” clinically relevant?

A
  • One of the criteria of diffuse midline glioma, H3 K27M altered
  • May occur in other gliomas not located in the midline (pilocytic astrocytoma and ependymoma)
35
Q

In what glioma type/s is “EGFR gene amplification” clinically relevant?

A
  • Common in glioblastoma, IDH-wildtype CNS WHO grade 4
  • If present in astrocytoma, IDH-wildtype CNS WHO grades 2 or 3, it is consistent with glioblastoma, IDH-wildtype CNS WHO grade 4
36
Q

In what glioma type/s is “CDKN2A/B homozygous deletion” clinically relevant?

A
  • Present in astrocytoma, IDH-mutant indicates poor prognosis and despite lack of microvascular proliferation and or necrosis they are grade 4.
37
Q

In what glioma type/s is “BRAF V600 mutation” clinically relevant?

A
  • Frequently present in pleomorphic xanthoastrocytoma, also in ganglioglioma and epitheloid glioblastoma
38
Q

In what glioma type/s is “ATRX mutation” clinically relevant?

A
  • Common in astrocytoma, IDH-mutant (not in oligodendroglioma) and diffuse hemispheric glioma, H3 G34–mutant
39
Q

What more is needed for a IDH1 wt diffuse glioma to be named “Glioblastoma IDH wt CNS WHO gr4”?

A

One or more of the following:
* microvascular proliferation
* Necrosis
* TERTp mutation
* EGFR gene amplification
*+7/-10

40
Q

What more is needed for a diffuse glioma, IDH1 mutant to be a Oligodendroglioma IDH mutant (XXXXX) CNS WHO grade 2/3?

A

ATRX retained and 1p/19 codeletion.

41
Q

What more is needed for a diffuse glioma, IDH1 mutant to be called “Astrocytoma IDH-mutant CNS WHO grade 2/3”?

A

ATRX mutation, P53 mutation

42
Q

What homozygous deletion can be found in an otherwise “Astrocytoma IDH mutant grade 2/3” that will make it an “Astrocytoma IDH mutant CNS WHO grade 4”?

A

CDKN 2A/B

43
Q

What is a diffuse glioma, IDH1 mutant, ATRX mutant/P53 mutant with microvascular proliferation and/or necrosis called?

A

Astrocytoma IDH mutant CNS WHO grade 4.

44
Q

WHO 2016 called “Glioblastoma, IDH mutant WHO grade IV” is now called

A

Astrocytoma, IDH mutant, CNS WHO grade 4.

45
Q

WHO 2016 called “Diffuse astrocytoma, IDH-wildtype, WHO grade II*” is now called

A

Glioblastoma, IDH-wildtype, CNS WHO grade 4

46
Q

WHO 2016 called “Diffuse astrocytoma, IDH-wildtype, WHO grade II*” is now called

A

Glioblastoma, IDH-wildtype, CNS WHO grade 4

47
Q

WHO 2016 called “Anaplastic astrocytoma, IDH-wildtype, WHO grade III*” is now called

A

Glioblastoma, IDH-wildtype, CNS WHO grade 4

48
Q

WHO 2016 called “Anaplastic oligodendroglioma, IDH-mutant and 1p/19q codeleted WHO grade III” is now called

A

Oligodendroglioma, IDH-mutant and 1p/19q codeleted, CNS WHO grade 3

49
Q

WHO 2016 called “Anaplastic astrocytoma, IDH-mutant, WHO grade III” is now called

A

Astrocytoma, IDH-mutant, CNS WHO grade 3

50
Q

Name (A-F) the new major groups of gliomas, glioneuronal and neuronal tumors

A

A. Adult type diffuse gliomas
B. Pediatric type diffuse low grade gliomas
C. Pediatric type diffuse high grade gliomas
D. circumscribed astrocytic gliomas
E. Glioneuronal and neuronal tumors
F. Ependymomas

51
Q

What is the most common adult intraaxial tumor?

A

Glioma, about 6/100.000

52
Q

How many % of gliomas recur away from original site?

A

10%

53
Q

Name the 3 main ways a glioma may spread

A
  1. through white matter
  2. Through CSF
  3. Rarely they spread systemically.
54
Q

Name 5 ways gliomas can spread through white matter

A
  1. corpus callosum
  2. cerebral peduncles
  3. internal capsule
  4. uncinate fasciculus
  5. interthalamic adhesion
55
Q

What type of spreading of glioma come from way through corpus callosum?

A
  1. through genu or body CC - bilateral frontal lobe involvement (butterfly glioma)
  2. through splenium of CC - bilateral parietal or occipital lobes
56
Q

What type of spreading of glioma come from way through cerebral peduncles?

A

midbrain involvement

57
Q

What type of spreading of glioma come from way through internal capsule?

A

encroachment of basal ganglion tumors into centrum ovale

58
Q

What type of spreading of glioma come from way through uncinate fasciculus?

A

simultaneous temporal and frontal tumours

59
Q

What type of spreading of glioma come from way through interthalamic adhesion?

A

bilateral thalamic gliomas

60
Q

What typen of glioma is most associated to cysts?

A

Pilocytic astrocytomas (circumscribed astrocytic glioma)

61
Q

How to differentiate cystic fluid from glioma from CSF

A

Its usually xanthocromic and clots when removed from the body.

62
Q

What does “H3 K27M” stand for?

A

Histone H3 K27M

63
Q

What does “MGMT” stand for?

A

methylation O6-methylguanine DNA methyltransferase

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