Histopathology - Neurooncology Flashcards

1
Q

Tumours of the PNS

Small nerves
Large nerves

A

Small nerves - neurofibromas

Large nerves - schwanommas

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2
Q

Commonest type of CNS tumours in

adults
children

A

adults - secondary (metastases)
o Well demarcated, solitary or multiple with surrounding oedema

children - primary

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3
Q

Commonest extra-axial and intra-axial tumour

A

extra-axial - meningioma

intra-axial - glial cell tumour

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4
Q

Commonest malignant vs non-malignant primary CNS tumour

A

malignant - GBM

Non-malignant - non-malignant meningioma

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5
Q

Where does glioblastoma originate from?

A

Astrocytes

It is a neuroepithelial tumour

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6
Q

Neurofibromatosis chromosomes and tumours

A

NF1 - Chr 17
neurofibroma, pilocytic astrocytoma, optic glioma

NF2 - Chr 22
Schwannoma, meningioma, ependymoma, astrocytoma

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7
Q

Grade of gliomas and radiotherapy vs chemotherapy

A

Radiotherapy - low grade, high grade, mets, benign tumours

Chemotherapy - high grade, lymphomas
temozolomide

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8
Q

WHO classification of brain tumours

A

Tumour type (histological type - derived from cell of origin or lineage of differentiation)

Grade - based on morphological/ histological criteria and predicted natural clinical behaviour (does not consider response to treatment)
 Grading tells us survival but does not tell us therapy response/disease spread/cell of origin
• Survival refers to the natural history of the tumour (i.e. if they are not treated)

Molecular profile - genetic profile, molecular markers

staging NOT used except for medulloblastoma

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9
Q

Which are the 3 gliomas

A

Astrocytes - astrocytoma (commonest)
oligodendrocytes - oligodendroglioma
ependyma - ependymoma

astrocytomas are more aggressive than oligodendroglioma

ependyma = the thin membrane of glial cells lining the ventricles of the brain and the central canal of the spinal cord.

(also
schwann cells
microglia
satellite cells)

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10
Q

What kind of tumour originated from Embryonal cells?

A

Medulloblastoma

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11
Q

Low grade vs high grade

A

low grade
grade 1 - benign, long term survival
grade 2 - >5y

high grade
grade 3 - <5y
grade 4 - <1y

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12
Q

Most common primary CNS tumour

A

Glioma

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13
Q

Which are the glial cells

A
Astrocytes
oligodendrocytes
ependymal cells
schwann cells
microglia
satellite cells
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14
Q

Mutations in diffuse glial tumours that are good prognostic factors and are associated with long term survival

A

IDH1/2 mutations

associated with a longer survival + a better response to chemo/radio

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15
Q

Gliomas in adults vs children

A

adults - diffuse gliomas, supratentorial, grades >=2, malignant, IDH1/IDH2, H3 mutations

children - circumscribed gliomas, posterior fossa, grades 1-2, rarely malignant, MAPK pathway mutations (BRAF (50%), NF1, FGFR1)

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16
Q

most common brain tumour in children

second most common brain tumour in children

A
Pilocytic astrocytoma (grade 1)
medulloblastoma (grade 4)
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17
Q

pilocytic astrocytoma buzzwords

location
mutation
MRI
Histopathology

A

circumscribed glioma
grade 1
most common child brain tumour
CEREBELLAR, optic-hypothalamic, brainstem

NF1
BRAF mutation (70%)

MRI - well circumscribed, cystic, enhancing lesion, little surrounding oedema

Histopathology
Piloid cell
Rosenthal fibres, granular bodies
Slow growing, low mitotic activity

18
Q

Oligodendroglioma buzzwords

A

20-40y
Long history of neurological signs (e.g. seizures)

cytology –> fried eggs –> round cells with clear cytoplasm

soft, gelatinous, calcified

better prognosis than astrocytoma
(slow growth - can be resected, good response to chemo/radio)

IDH1/2 mutation + co-deletion of 1p/19p

19
Q

How does astrocytoma present on an MRI

A

Non enhancing lesion

20
Q

Histology of astrocytoma

A

low mitotic activity
low cellularity
no vascular proliferation or necrosis

21
Q

where is astrocytoma found in

adults
children

A

adults - cerebral hemisphere

children - brainstem, cerebellum

22
Q

Most common glioma

A

GBM

23
Q

Most common primary CNS tumour

A

GBM

24
Q

Most aggressive glioma in adults

A

GBM

25
Q

How can a GBM have an IDH1 mutation?

A

90% of cases of GBM occur denovo and have wildtype IDH(poor prognosis)

10% occur secondary to astrocytoma progression and have the IDH mutation (positive prognostic factor)

26
Q

GBM

MRI
Histology

A

MRI
HETEROGENOUS mass
enhancing post-contrast

histology
high cellularity
high mitotic activity
microvascular proliferation (neoangiogenesis)*
necrosis
  • abnormal blood vessels –> typical of GBM
27
Q

1st and 2nd most common CNS tumours in adults

A

1 glioma

2 meningioma

28
Q

what kind of tumour is meningioma?

A

extra-axial

29
Q

Meningioma

MRI
Histology

A

MRI
Extra-axial, isodense, contrast-enhancing

Histology
Psammoma bodies(round collection of calcium)
calcification

30
Q

Most useful predictor of recurrence

A

Grading

31
Q

Mitotic activity

grade 1
grade 2
grade 3

A

mitotic activity per HPF (high power field)

grade 1 <4
grade 2 4-20
grade 3 >20

32
Q

How do we determine origin of brain mets?

A

Immunohistochemistry

33
Q

Where is metastatic brain disease usually found and why?

A

• Located at grey/white matter junction +/or leptomeningeal disease (cortical – subcortical junction)

o As structure of cerebral blood vessels changes at this point (become smaller as they enter the white matter) -> neoplastic emboli tend to get stuck at this level and then start growing

34
Q

Most frequent cancers causing mets to the CNS

A

Lung
Breast
Melanoma
Renal

35
Q

Where does medulloblastoma originate from

A

Neuroepithelial cells or neuronal precursors of the cerebellum/ dorsal brainstem

Derived from periventricular tissue around the 4th ventricle

36
Q

Medulloblastoma histopathology

A

Blue tumour “small blue round cell”*

Large hypochromic nuclei
Homer Wright rosettes*
https://www.bing.com/images/search?q=homer+wright+rosettes&form=HDRSC2&first=1&tsc=ImageHoverTitle

grow in sheets
express neuronal markers (i.e. very little differentiation) - synaptophysin, GFAP, Ki67

*features of primitive cell lines/primitive neuronal differentiation

37
Q

What is methylome profiling and where is it used?

A

o Most tumours have characteristic patterns of DNA methylation of CpG islands

o The methylation signature is stable + reflects the tumour cell of origin –> Give information on tumour type

 Helpful for atypical cases, rare entities, small biopsies, subtyping

 Not used for grade/progression or targeted therapy
 does not consider the histology/ does not require histological correlation

38
Q

• Buzzwords

o NF2
o Ventricular tumour, hydrocephalus
o Indolent, childhood
o Soft, gelatinous, calcified

A

• Buzzwords
o NF2 – meningioma

o Ventricular tumour, hydrocephalus – ependymoma

o Indolent, childhood – pilocytic astrocytoma

o Soft, gelatinous, calcified - oligodendroma

39
Q

a. Seizure following 2 weeks of left arm and leg weakness
b. MRI showing heterogeneous enhancing right frontal lesion, started on steroids
c. Partial response to steroids with improved dexterity of the left arm and leg
d. A tumour was partially resected

e. Glioblastoma (WHO grade IV)
f. Oligodendroglioma (WHO grade II)
g. Metastatic carcinoma
h. Diffuse astrocytoma (WHO grade II)

A

i. Answer – E

MRI showing HETEROGENOUS ENHANCING right frontal lesion, started on steroids

40
Q
  • year-old boy
    a. Had headache and vomiting in the morning for 2 weeks
    b. Symptoms worsened and the vision became blurred
    c. Fundoscopic exam: papilledema
    d. MRI showing cystic cerebellar lesion  tumour was removed

e. Glioblastoma (WHO grade IV)
f. Medulloblastoma (WHO grade IV)
g. Oligodendroglioma (WHO grade II)
h. Pilocytic astrocytoma (WHO grade I)

A

i. Answer - H
Histology is of a low grade glial tumour, not a blue tumour (medulloblastoma)

d. MRI showing CYSTIC CEREBELLAR lesion