Neurooncology Flashcards

1
Q

What are the most common primary CNS tumours? (Benign and malignant)

A

Mostly: Benign
40% Bening Meningioma
17% Benign Pituitary

Malignant: 30%

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2
Q

What are 3 Common Familial CNS tumour syndromes and what types of CNS tumours do they cause?

A

Neurofibromatosis 1: neurofibroma, astrocytoma
Neurofibromatosis 2: schnwannoma, meningioma
Von Hippel Lindau: Hemangioblastoma

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3
Q

What primary brain tumours usually benefit most from Radiotherapy?

A
  1. Low and high grade gliomas, metastases, some benign
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4
Q

What primary brain tumours are usually treated by chemotherapy?

A

Mainly high grade gliomas (temozolomide) and lymphomas

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5
Q

Where do extra-axial brain tumours originate from?
Do they tend to be bening or malignant?

A

Cranium
softtissue
meninges
nerves
[BENIGN]

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6
Q

Where to intra-axial brain tumours usually originate from?
Do they tend to be belign or malignant

A

glial
neurons
neuroendocrine cells

Usually MALIGNANT, but rarely metastasise outside the CNS

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7
Q

What systemic tumours like to metastasise to the brain?

A

lung, breast, malignant melanoma and renal cell

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8
Q

What is the radiological appearance of CNS metastasis on a CT head?

A

Well demarcated, solitary or multiple with surrounding oedema

Can be multiple

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9
Q

What are infratentorial signs and symptoms of brain tumours?

A
  1. Ataxia
  2. Long tract signs - spasticity and hyperreflexia
  3. Cranial nerve palsies

Located below the tentori cerebellum –> Cerebellum + Brain stem

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10
Q

What are primary brain tumours derived from astrocytes called?

A

Astrocytomas

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11
Q

What are primary CNS tumours called derived from oligodendrocytes?

A

oligodendroglioma

BUZZWORD
“fried-egg”appearance

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12
Q

What are primary CNS tumours called derived from empendyma?

A

ependyoma

BUZZWORD
ventriculartumour hydrocephalus

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13
Q

What are primary CNS tumours called derived from Embryonal cells?

A

Medullablastoma

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14
Q

How does the Grading of primary CNS tumours work?
What predictions are they based on?
What are they?

A

Based on natural clinical behaviour (without treatment - with treatment can be different)

Grade 1–benign
Grade 2–more than 5 years survival
Grade 3–1- 5 years survival
Grade 4 – less than 1 year survival

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15
Q

Which CNS cells are classified as glial cells?

A

Astrocytes + Oligodendrocytes

(Macroglia)
Ependymal cells
Tanycytes

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16
Q

What are Pilocytic Astrocytomas?
What grade are they usually and what epidemiology?

A

Primary CNS tumours derived from Astrocytes

Usually in children (0-20 years)
and Grade 1 (benign)

17
Q

What are histological features of pilocytic astrocytomas?

A

Piloid “hairy” cells
Rosenthal fibres
Slow mitotic divisions

18
Q

What site are pilocytic astrocytomas usually occur?

A

Often cerebellar
Optic-hypothalamus lesions (In Neurofibromatosis type 2)
Brainstem

19
Q

What genetic mutation is associated with Pilocytic astrocytomas?

A

BRAF mutation in 70% of tumours

20
Q

What are Diffuse Gliomas?
What grade are they usually and what epidemiology?

A

Primary CNS tumours derived from Glial cells, usually Grade 2-3

Include Astrocytomas and Oligodendrogliomas

Age group: 20-40

21
Q

What genetic mutations are diffuse Gliomas associated with?

How does this change the prognoisis?

A

IDH mut. is associated with longer survival and better response to chemo and radiotherapy

22
Q

What are histological features of astrocytomas (diffuse Gliomas)?

A

Low-moderate cellularity

Low mitotic activity

No vascular proliferation
(+ IDH mutant can be detected by immunocytochemically)

23
Q

What is the prognosis of Astrocytomas/ Diffuse gliomas?

A

Depends on Grade (2-4)
But overall you would expect progression over time

IDH mutation associated with better outcome + survival

24
Q

What are Diffuse Glioblastoma multiforme?
What grade are they usually and what epidemiology?

A

Primary Mailgnant CNS tumour

Very aggressive (Grade 4) but most frequent primary CNS tumour in adults
(Median survival 8 months)

Age usually 50+

25
Q

What is the site distribution of glioblastoma multiforme?

A

Usually supratentorial (so affecting the cerebral hemispheres

26
Q

What genetic mutations are associated with Glioblastoma multiforme?

A

IDH wild type
other mutations can occur, but are less imporant

27
Q

What histological signs would you see on a glioblastoma multiforme?

A

High cellularity
High mitotic activity
Microvascular proliferation Necrosis

28
Q

What is the epidemiology of meningiomas?

A

Usually >40, incidence increases with age

29
Q

What grade is a CNS meninioma?
What is the recurrence rate?

A

Variable Grade 1-3

80% benign (1) and <25% recurrentce
20% Grade 2, atypical and 25-50% recurrence
1% Grade 3, malignant with 50-90% recurrence

30
Q

Where do meningiomas originate from?

A

Can originate from any site of craniospinal axis

Originate from meningothelial cells of the arachnoid mater

31
Q

What histological features do meningiomas usually show?

A

Psammoma bodies (calcifications)

Mitotic activity determines grading

(<4 grade 10
4-20grade 2
>20 grade 3)

32
Q

Where are secondary brain metastasis usually located?

A

At grey/white matter junction and/or leptomeningeal (inner 2 layers of meninges) diasese

33
Q

What cell types does a Medulloblastoma originate from?
What grade is it usually?

A

Embryonal tumour - originates from neuroepithelial cells/neuronal precursors of the cerebellum or dorsal brainstem

Often/usually Grade 4, however outcome have improved with radio-chemotherapy and subtype stratification

34
Q

What is the epidemiology of medulloblastomas?

A

2nd most common brain tumour in children after Astrocytomas

(But still very rare -2/1.000.000)

35
Q

What radiological appearance is associated with glioblastoma multiforme?

A

On MRI:

heterogenous,
enhancing post contrast