Histopathology 14 - Neuro-oncology Flashcards

(48 cards)

1
Q

Explain the difference between extra axial and intra-axial brain tumours

A

Extra-axial (coverings): (grade1)

tumours of bone, cranial soft tissue, meninges, nerves and metastatic deposits

Intra-axial (parenchyma): (grades 2-4)

Derived from normal cell populations of CNS (glia, neurons, vessels, connective tissue)

Derived from other cell types (metastases, lymphomas, germ cell tumours)

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2
Q

What does tumour grading tell us?

A

survival - NOT therapy response, disease spread or cell of origin

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3
Q

What is the most common form of primary brain tumour?

A

Glial tumours - gliobastoma multiforme

(Astrocytes, Oligodendrocytes, Ependymal cells, Schwann cells, Microglia, Satellite cells)

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4
Q

What are the 2 broad subtypes of glial tumours, and in which age groups are they typically seen?

A

Diffuse (adults) or circumscribed (children)

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5
Q

Diffuse vs circumscribed gliomas

A

diffuse gliomas

grades ≥II, adults, supratentorial, malignant progression

diffuse astrocytoma, GBM, oligodendroglioma

circumscribed gliomas

grades I-II, children, often posterior fossa, rare malignant transformation

pilocytic astrocytoma, pleomorphic xanthosastrocytoma, subpendymal giant cell astrocytoma, ependyoma

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6
Q

What grade are diffuse vs circumscribed glial tumours?

A

d: ≥2
c: 1 or 2

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7
Q

What mutation is associated with diffuse vs circumscribed gliomas?

A

Diffuse - IDH1/2 in 30% (positive prognostic factor - longer survival and better response to chemo and radiotherapy) + H3 (1%)

Circumscribed - MAPK pathway mutations (BRAF, NF1, FGFR1)

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8
Q

Describe the progression of all diffuse glial cell tumours

A

Cannot be removed surgically, so all progress to glioblastoma within 10 years

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9
Q

Which gene mutation is associated with diffuse glial cell tumour prediliction?

A

IDH gene

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10
Q

Epidemiology of diffuse astrocytoma

A

Patients 20-40yo

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11
Q

What would you see on MRI in diffuse astrocytma?

A

Cerebral hemispheres (adults), cerebellum (children)

MRI: T1 hypointense, T2 hyperintense, non-enhancing lesion

Low choline: creatinine ratio at MR-spectroscopy

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12
Q

describe the epidemiology of glioblastoma multiforme

A

>50y, increasing incidence with age

10% of cases secondary to astrocytoma (progression) - IDH mutation ( pos prognosis factor)

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13
Q

What would you see on MRI in GBM?

A

cerebral hemispheres

heterogenous, enhancing post-contrast

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14
Q

describe the histological features of glioblastoma multiforme

A

high cellularity, high mitotic activity, microvascular proliferation (neoangiogenesis), necrosis

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15
Q

What is the most common type of circumscribed glial tumour?

A

Pilocystic adenoma

Most common child brain tumour (20% CNS tumours <14yo)

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16
Q

Which brain tumour is an indolent CNS tumour of childhood?

A

Pilocystic astrocytoma

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17
Q

Mutation of which gene is associated with circumscribed glial tumours?

A

BRAF gene mutation

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18
Q

What would see on MRI in pilocystic astrocytoma?

A

MRI: cerebellar; well circumscribed, cystic, enhancing

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19
Q

What would you see on histology in pilocystic astrocytoma?

A

Piloid (hairy) cell

Rosenthal fibres and granular bodies

Slow growing with low mitotic activity

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20
Q

How can glioblastoma and diffuse astrocytoma be differentiated on CT?

A

Glioblastoma = enhancing
Diffuse astrocytoma = non-enhancing

21
Q

Which mutation is most associated with pilocystic astrocytoma?

A

BRAF mutation in 70%

22
Q

Describe the epidemiology of meningiomas

A

Rare in patients <40yo

increasing incidence with age

23
Q

Which genetic syndrome is associated with meningioma?

A

Neurofibromatosis type 2

24
Q

What tumour type typically takes part in microscopic brain invasion

A

meningiomas

Attaches to meninges but does not typically invade, just displaces brain matter

If they do invade, it is often a micro-invasion (as pictured)

25
In which type of brain tumours are psamomma bodies found
meningiomas - globules on histology
26
Which 3 types of cancer are most likely to metastasise to the brain?
Lung Breast Malignant melanoma
27
What is a common site for brain Ca mets?
grey-white junction structure of cerebral blood vessels changes at this point (become smaller as they enter white matter) Therefore, neoplastic emboli tend to get stuck at this level and then start growing
28
Describe the features of medulloblastoma (grade 4)
embryonal tumour originates from neuroepithelial precursors of cerebellum/dorsal brainstem Cerebellum Rare; but 2nd most common brain tumour in children
29
Describe the histological features of medulloblastoma
HIGH GRADE, POORLY DIFFERENTIATED NEUROEPITHELIAL TUMOUR “Small blue round cell” tumour (i.e. blastoma/primitive cell line – another = Wilm’s tumour) Expression of neuronal markers (very little differentiation) – i.e. synaptophysin **Homer-Wright rosettes** are a feature of primitive neuronal differentiation
30
What is the only known environmental risk factors for neuro tumours?
radiotherapy to head and neck → meningiomas, rarely gliomas
31
What type of tumour is being described by "a ventricular tumour that presents with hydrocephalus"?
Ependyoma
32
Describe the signs of symptoms of supratentorial vs subtentorial tumours
**supretentorial** * focal neurological deficit * seizures * personality change **subtentorial** * cerebellar ataxia * long tract signs * cranial nerve palsy
33
Recall the order in which imaging should be performed in CNS tumour investigation
1. CT 2. MRI (T1 and without contrast) 3. MR spectroscopy for gliomas (useful prognostically)
34
Which gene mutation is always seen in oligodendroglioma?
IDH gene
35
What is the WHO grade of oligodendroglioma?
Stage 2 - 3
36
What is the pathognemonic histological finding in oligodendroglioma?
Fried egg cells
37
How is oligodendroglioma most often managed?
Usually appropriate for surgical excision as often well-circumscribed
38
What must be investigated in the case of multiple meningiomas?
Neurofibromatosis type 2
39
What is the most important factor to assess when grading meningiomas?
Mitotic activity
40
Where do medulloblastomas always form?
In cerebellum near 4th ventricle
41
What is the pathognemonic histological finding in medulloblastoma?
Very poorly differentiated 'small round blue cells'
42
Which subtype of medulloblastoma has the best prognosis?
WNT-activated subtype
43
What is the basis of epigenetic profiling in CNS tumours?
DNA methylation of CpG islands
44
what is the most frequent brain tumour in adults?
mets
45
what is the most frequent brain tumour in children
pilocytic astrocytoma
46
**45 y/o headache, prev pulmonary lobectomy, CT shows frontal lesion. diagnosis?**
mets
47
5 y/o headache, vomiting and papilledema. MRI shows cystic cerebellar lesion. diagnosis?
pilocytic astrocytoma
48
70 y/o seizure following left arm and leg weakness. MRI shows heterogeneous enhancing R frontal lesion. Diagnosis?
glioblastoma