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RADT317 - Brain > High grade gliomas > Flashcards

High grade gliomas Flashcards

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1
Q

What percentage of gliomas to astrocytomas account for?

A

33% of all gliomas

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2
Q

What percentage of CNS tumours do astrocytomas account for?

A

7-10%

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3
Q

What are most astrocytomas graded as?

A

1-4

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4
Q

What are grade 3 and 4 astrocytomas visually?

A

Almost indistinguishable

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5
Q

What are grade 1 astrocytomas labelled as?

A

Pilocytic

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6
Q

What age do group grade 1 (pilocytic) astrocytomas arise?

A

Children and young adults

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7
Q

Where do grade 1 (pilocytic) astrocytomas mainly arise?

A

Posterior fossa

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8
Q

What is the behaviour of grade 1 (pilocytic) astrocytomas?

A

Slow growing - may stop or regress

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9
Q

What is the grade 1 (pilocytic) astrocytoma survival percentage?

A

80-90%

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10
Q

What improves grade 1 (pilocytic) astrocytoma prognosis?

A

Complete resection improves prognosis. Recent understanding of the biology of the tumour means that there may be alternative biological therapies.

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11
Q

What are other sites of grade 1 (pilocytic) astrocytoma other than posterior fossa?

A

Cerebrum, optic nerve and chiasm, thalamus and basal ganglia brain stem and spinal cord (occasionally SCF)

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12
Q

What do the majority of grade 1 (pilocytic) astrocytoma tumours never become?

A

They never become less well differentiated and more malignant

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13
Q

What are grade 2 astrocytomas labelled as?

A

Diffuse

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14
Q

What age do group grade 2 (diffuse) astrocytomas arise?

A

Young adults

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15
Q

Where do grade 2 (diffuse) astrocytomas mainly arise?

A

Cerebrum, brain stem and spinal cord

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16
Q

How do grade 2 (diffuse) astrocytomas present?

A

As seizures

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17
Q

What is the median postoperative survival of grade 2 (diffuse) astrocytomas?

A

5-8 years

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18
Q

What do grade 2 (diffuse) astrocytomas evolve to be?

A

Malignant

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19
Q

What role do RT and chemo have in the management of grade 2 (diffuse) astrocytomas?

A

Controversial

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20
Q

What are grade 3 astrocytomas labelled as?

A

Anaplastic (“diffusely infiltrating”)

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21
Q

What age do group grade 3 (anaplastic) astrocytomas arise?

A

Middle age

22
Q

What is the median postoperative survival of grade 3 (anaplastic) astrocytomas?

A

2-4 years although there are people who can live much longer

23
Q

What do grade 4 (anaplastic) astrocytomas evolve to be?

A

Almost always fatal. At the later stages it is thought to transform to glioblastoma.

24
Q

What are grade 4 astrocytomas labelled as?

A

IDH mutant

25
Q

What were grade 4 (IDH Mutant) astrocytomas labelled as prior to the WHO classification of CNS tumours 2021?

A

Considered Glioblastoma prior to the ‘WHO classification of CNS tumours -5th Ed (2021)

Classification changed based on the presence of IDH mutation – Glioblastoma now considered separate entity.

26
Q

What age do group grade 4 (IDH Mutant) astrocytomas arise?

A

Generally younger presentation than glioblastoma

27
Q

What is the male:female ratio of grade 4 (IDH Mutant) astrocytomas?

A

M:F around 1.5

28
Q

What is the management of grade 4 (IDH Mutant) astrocytomas?

A

The same as glioblastoma

29
Q

What is the survival in years of grade 4 (IDH Mutant) astrocytoma?

A

5 year survival – 3.6 yrs. – better than glioblastoma

30
Q

What percentage of pre-WHO 5th Ed of cases would’ve been classified as glioblastoma?

A

Around 10% of pre-WHO 5th Ed of cases=G4 IDH Mutant (would have been classified as glioblastoma prior to this)

31
Q

What are grade 4 glioblastomas labelled as?

A

IDH-Wildtype

32
Q

What age do grade 4 (IDH Wildtype) glioblastomas arise?

A

Any age but most common in those 70 +

33
Q

Where do grade 4 (IDH Wildtype) glioblastomas mainly arise?

A

Cerebrum
Basal ganglia
Rarely arises in the cerebellum
A small percentage cross corpus callosum when it is described as the “butterfly tumour”

34
Q

How does grade 4 (IDH Wildtype) appear?

A

Macroscopic appearance – grey mass with areas of haemorrhaging, yellow necrosis and sometimes cysts.

Appear to have a capsule – this is an artefact of rapidly growing tumour and compressed brain.

Adjacent brain is swollen and oedematous

Spreads along white matter tracts..

Migratory cells are found many cm from the main body of the tumour

Tumour appears to be multi-focal but are actually from one site

Microvascular proliferation and necrosis are the hallmarks of glioblastoma.

Glioblastoma is recognisable radiologically

35
Q

What is glioblastoma (IDH Wildtype) in terms of malignancy?

A

Most malignant form of glioma. It is poorly differentiated and highly invasive. It has areas of necrosis and microvascular proliferation

Diffuse astrocytic tumour
Necrosis
Microvascular proliferation
EGFR gene amplification

Formed de novo.

36
Q

What does MGMT stand for?

A

Methylguanine DNA Methyltransferase

37
Q

What are the advantages of MGMT Methylation Promotor?

A

Generally longer survival than those without MGMT methylation promotion

Positively impacts the efficacy of Temozolomide
?Offer temozolomide to patients without promotion

38
Q

What are the advantages of Isocitrate Dehydrogenase - IDH1 & 2 Gene Mutations?

A

Patients whose tumours express IDH mutations have improved survival compared to wild-type IDH

90% Wild-Type
Tumour formed as a Glioblastoma from the beginning

10% Mutated
Tumour was previously a lower grade tumour

39
Q

What does IDH stand for?

A

Isocitrate Dehydrogenase

40
Q

What are the 3 forms of glioblastoma?

A

Giant cell glioblastoma
Gliosarcoma
Epithelioid glioblastoma

41
Q

Who does glioblastoma most likely occur?

A

Occurs in older people
No prior history of tumour
Poor prognosis

42
Q

How does glioblastoma appear?

A

Grey masses
Areas of haemorrhage
Yellow necrosis
Cysts
Never encapsulated (appear to have capsule – this is an artefact of rapidly growing tumour and compressed brain)
Tissue adjacent to tumour oedematous
Oedema located around white matter
Offers a route for satellite cells to migrate from the main body of the tumour
This can make the tumour appear multifocal

43
Q

What is the management of glioblastoma?

A

Biopsy to confirm diagnosis

Removal of maximum volume of tumour possible (maximal safe resection (MSR)

Radiotherapy

Chemotherapy (TMZ)

44
Q

What does WHO grade oligodendroglioma as?

A

Grade 2

45
Q

What percentage are oligodendrogliomas?

A

5-10%

46
Q

What is the peak age for oligodendrogliomas?

A

30-50 years old

47
Q

Where do oligodendrogliomas arise?

A

Arise in white matter:
Frontal, parietal and temporal lobes
occasionally arises in the spinal cord
Very rarely arises in the cerebellum

48
Q

How do oligodendrogliomas present?

A

Slow growing

Patient often describes long history of seizures.

CT, MRI and Plain skull x-rays often reveal calcification (~50% cases)

Long standing tumours may cross corpus callosum – even when low grade

Unlike glioblastoma – occasionally high grade oligodendroglioma can do unaccountably well

49
Q

How do oligodendrogliomas appear on a CT?

A

Often areas of calcification evident

50
Q

Do oligodendrogliomas respond to chemo?

A

Yes – PVC (procarbazine, lomustine, vincristine)

RADT317 - Brain (10 decks)

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