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

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3
Q

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

A

Diffuse Gliomas

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4
Q

Give 2 examples of Glial tumours in adults ?

A

Astrocytoma

Oligodendrogliomas

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5
Q

What’s the most common glial tumour in children ?

A

Pilocytic astrocytoma

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6
Q

What are the typical histological features of Pilocytic Astrocytomas ?

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

IDH2

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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

The 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

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13
Q

List 3 typical features of medulloblastoma on histology ?

A

Small blue round cells
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)

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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

17
Q

List 3 features of von hippel-Lindau syndrome ?

A
  • Hemangioblastoma
  • Renal cysts
  • Phaeochromocytoma
18
Q

List 5 features of Tuberous sclerosis

A
  • Epilepsy
  • Learning difficulties
  • Skin: Ash leaf spots, angiofibromas, shagreen patches
  • Neuro: Giant cell astrocytoma, cortical tubers
  • Renal: Angiomyolipomas
  • Cardio: Rhabdomyomas
19
Q

List 3 features of Neurofibromatosis type 2

A
  • Bilateral Schwannomas -Affect CNVIII causing hearing loss, tinitus
  • Meningiomas
  • Cataracts
20
Q

List 5 features of Neurofibromatosis type 1

A
  • Axillary freckling
  • Cafe au lait spots
  • Neurofibromas
  • Lisch nodules
  • Optic glioma
21
Q

difference between extra-axial and intra-axial CNS tumours

A

extra-axial = coverings
tumours of bone, cranial soft tissue, meninges, nerves and metastatic deposits

intra-axial = parenchyma
glia, neurons, vessels, connective tissue
derived from other cell types (meets, lymphomas)

22
Q

usual inheritance of CNS tumours

A

autosomal dominant

23
Q

presentation of CNS tumours

A

supratentorial = focal neurological deficit, seizures, personality changes

subtentorial = cerebellar ataxia, long tract signs, cranial nerve palsy

24
Q

what chemo is used in high grade gliomas

A

temozolamide

25
Q

features of glial tumours

A
IDFFUSE INFILTRATION 
adults 
grades >2
astrocytomas or oligodendroglioma 
IDH1/2 mutation 
CIRCUMSCRIBED GLIOMAS 
children 
grades 1-2
pliocytic astrocytomas
MAPK mutation
26
Q

features of pliocytic astrocytomas

A
grade 1
children 
NF 1
MRI - well circumscribed, cystic, enhanced lesion 
cerebellar 
piloid cells and rosenthal fibres 
BRAF mutation
27
Q

features of astrocytomas

A
grade 1-4 
cerebral hemispheres common sites 
malignant progression 
become glioblastomas 
IDH2 mutation
28
Q

features of glioblastoma multiforme

A

grade 4
>50 yrs
microvascular proliferation and necrosis
wildtype IDH mutation

29
Q

features of oligodendromas

A

grade 2-3
round cells with clear cytoplasm (fried egg)
IDH 1/2 mutations, co-deletion of 1p/19q
good prognosis

30
Q

features of meningiomas

A

grade 1-3
can be multiple in NF2
focal symptoms
brain invasion

31
Q

featres of medulloblastomas

A
grade 4 
embryonal tumour 
cerebellum always 
2nd most common brain tumour in children 
wright rosettes and synaptophysin
32
Q

features of CNS mets

A

multiple
lung, breast, melanoma
poor prognosis
seen at grey-white junction

33
Q

what can be used in future to classify CNS tumours

A

look at different patterns of DNA methylation