Histopathology 18: Neuro-oncology Flashcards

1
Q

List 4 genetic syndromes responsible for primary CNS tumours ?

A

Neurofibromatosis 1
Neurofibromatosis 2
Tuberous Sclerosis 1
Tuberous Sclerosis 2
Turbot’s syndrome

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2
Q

Outline the WHO grading of CNS tumours ?

A

Grade 1 : Benign, long-term survival
Grade 2 : causes death in > 5 years
Grade 3 : causes death in < 5 years
Grade 4 : causes death in < 1 year

NB Grade I and II are considered LOW grade

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3
Q

What type of tumours are the most common primary CNS tumours ?

A

Diffuse Gliomas (glial cells)

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4
Q

Give 2 examples of Glial tumours in adults ?

A

Astrocytoma
Oligodendrogliomas

NB diffuse = show a very infiltrative type of growth (become more malignant over time)

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5
Q

What’s the most common glial tumour in children ?

A

Pilocytic astrocytoma (NB this is a circumscribed glioma, not diffuse)

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6
Q

What are the typical histological features of Pilocytic Astrocytomas ? (2)

A

Piloid (hairy) cells
Rosenthal fibres

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7
Q

Which tumours do astrocytomas turn into after about 5-7 years ?

A

Glioblastomas

(It is a Grade 4 astrocytoma)

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8
Q

What mutation is most commonly seen in astrocytomas ?

A

IDH 1/2

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9
Q

Describe a typical feature of oligodendroglioma on histology ?

A

Fried eggs - Round cells with clear cytoplasm

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10
Q

Which genetic syndrome is associated with meningiomas ?

A

Neurofibromatosis 2

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11
Q

Where are medulloblastomas always found ?

A

cerebellum

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12
Q

List some symptoms of medulloblastoma ?

A

ICP symptoms- morning headaches, nause and vomiting, blurry vision

Loss of balance and coordination

Abnormal eye movements

NB cerebellum affected

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13
Q

List 3 typical features of medulloblastoma on histology ?

A
  • Small blue round cells (hyperchromatic nuclei with NO cytoplasm)
  • Homer-Wright rosettes
  • Neuronal marker Synaptophysin positive
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14
Q

Where are metastatic brain tumours most commonly found ?

A

The grey-white junction (where grey and white matter meet)

  • This is because the structure of the cerebral blood vessels change at this point (becoming smaller as they enter the white matter)
  • Therefore, neoplastic emboli tend to get stuck at this level and then start growing

NB another site = meninges

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15
Q

A ventricular tumour presenting with hydrocephalus.

Most likely diagnosis ?

A

Ependymoma

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16
Q

A soft gelatinous, calcified tumour.

Most likely diagnosis ?

A

Oligodendroma

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17
Q

usual inheritance of CNS tumours

A

autosomal dominant

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18
Q

presentation of CNS tumours

A
  • Intracranial Hypertension: headache, vomiting, change in mental state
  • supratentorial = focal neurological deficit, seizures, personality changes
  • subtentorial = cerebellar ataxia, long tract signs, cranial nerve palsy
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19
Q

what chemo is used in high grade gliomas

A

temozolamide

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20
Q

Tumour arising from Embryonal cells

A

medulloblastoma (2nd most common in children)

21
Q

Most common genetic syndrome related to brain tumours

A

Neurofibromatosis

22
Q

% of brain tumours due to genetics

A

<5%

23
Q

Genetics in Diffuse gliomas

A

IDH 1/2 mutation

24
Q

Genetics in Circumscribed Gliomas

A

MAPK pathway mutation (BRAF)

25
Q

Pilocytic astrocytoma found in which condition and in which group of pts?

A

NF1 - CHILDREN

26
Q

Astrocytoma location in brain

A

Often CEREBELLAR

27
Q

Mutation in Pilocytic Astrocytoma

A

BRAF Mutation (KIAA1549-BRAF fusion)

28
Q

Main site for Diffuse Astrocytoma

A

Cerebral hemispheres (see on MRI)

29
Q

Most aggressive type of glioblastoma

A

de novo glioblastoma

30
Q

Mutation in Diffuse Astrocytoma

A

Mutation in IDH1/2

31
Q

Effect of IDH mutation in Glioblastoma Multiforme

A

PROTECTIVE

  • IDH mutation is in astrocytoma, and 10% of glioblastoma arises from astrocytma (so secondary glioblastoma)
  • So protective in that it is not as bad a primary glioblastoma (without IDH mutation) as this is FAR MORE aggressive
32
Q

Name when glioblastoma spreads to other hemisphere

A

butterfly tumour

33
Q

Type of necrosis that can be seen in Glioblastoma Multiforme

A

pallisadian necrosis

  • there is necrosis surrounded by palisade tumour cells
34
Q

most frequent brain tumour in adults

A

Metastatic carcinoma

35
Q

most frequent brain tumour in children

A

Pilocytic astrocytoma

36
Q

What does the tumour grade tell us?

A

Survival - important prognostic factor

37
Q

24 y/o with long history of neurological signs (usually seizures). Which brain tumour?

A

Oligodendroglioma

  • Those between 20-40 years
  • get long history of neurological signs (usually seizures)
38
Q

Mutation in almost 100% cases of Oligodendroglioma

Diagnostic mutation

A

IDH1/2 mutation

Co-deletion 1p/19q (NB this mutation has better prognosis if present)

39
Q

Which has better prognosis, Oligodendroglioma or astrocytomas?

A

Oligodendroglioma (LESS aggressive and do not progress to a higher grade as quickly)

40
Q

What is the major change in the last WHO (2016) CNS tumour classification?

A

Incorporation of genetic profile

41
Q

Which mutation identifies the mutation diffuse astrocytic tumours with a better prognosis?

A

IDH mutation

42
Q

Most common, and 2nd most common, primary intracranial tumours

A
  • Most: gliomas
  • 2nd: Meningioma
43
Q

Where can you find Meningioma

A

anywhere in the craniospinal axis

44
Q

Condition where you get multiple meningioma

A

NF2

45
Q

Grade 2 meningioma is what?

A

When there is brain invasion

46
Q

Mx of Meningiomas that are grade ≥ II

A

radiotherapy as well as surgery

47
Q

Important aspect of determining grade of meningioma

A

Mitotic activity

  • Grade is based on mitoses/ 10 high power fields
48
Q

2nd most common tumour in kids

A

Medulloblastoma

49
Q

Prognosis of different classifications of medulloblastomas

A
  • WNT-activated – VERY GOOD prognosis (often cured with radio/chemotherapy)
  • SHH-activated – Intermediate prognosis
  • Non-WNT/non-SHH – VERY AGGRESSIVE, POOR prognosis

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