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Kat's Pathology > Neuro-Oncology > Flashcards

Neuro-Oncology Flashcards

1
Q

How are CNS tumours classified?

A

Primary - tumours that originated within the CNS

Secondary (metastases) - 10 times more frequent than primary tumours in adults

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2
Q

What is the most common form of neurological tumour

A

Secondary metastases

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3
Q

What is the second most common cancer in children

A

CNS tumours

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4
Q

How common are CNS tumours in adults

A

1-2% of all cancers

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5
Q

How are CNS tumours classified by location (2)

A

Extra-axial (coverings) - tumours of bone, cranial soft tissue, meninges and nerves.
Intra-axial (parenchyma) - derived from the major normal cell populations of the CNS (glia, neurons, vessels, connective tissue)
OR
Derived from other cell types (metastases, lymphomas, germ cell tumours)

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6
Q

What are the cells of origin of primary CNS tumours (7)

A 
Neurons. 
Astrocytes. 
Oligodendrocytes. 
Ependyma. 
Choroid plexus epithelium. 
Meningothelial cells. 
Embryonal cells.
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7
Q

What conditions have a genetic predisposition to CNS tumours (9)

A 
Neurofibramatosis 1
Neurofibramatosis 2
Tuberous Sclerosis 1
Tuberous Sclerosis 2 
Turcot's syndrome 
Li-Fraumeni 
Cowden syndrome 
Gorlin syndrome 
Von Hippel Lundau
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8
Q

What CNS tumours are associated with NF1 (2)

A

Neurofibroma

Pilocytic astrocytoma

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9
Q

What CNS tumours are associated with NF2 (2)

A

Schwannoma

Meningioma

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10
Q

What CNS tumours are associated with TS (2)

A

Hamartomas

Sega

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11
Q

What CNS tumours are associated with VHL

A

Hemangioblastoma

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12
Q

What is the only known environmental risk factor for CNS tumours

A

Ionising radiation

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13
Q

What are the signs of a supratentorial CNS tumour (5)

A 
Focal neurological deficit 
Seizures 
Headache 
Change in mental status 
Personality changes
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14
Q

What are the signs of a subtentorial CNS tumour (2)

A

Cerebellar ataxia
Long tract signs
Cranial nerve palsy

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15
Q

What imaging is used to assess CNS tumours (6)

A

CT
MRI

MR-Spectroscopy
Perfusion MRI
Functional MRI
PET-Scan

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16
Q

What is the purpose of neuroimaging (5)

A 
Assess tumour type 
Guide resection and biopsies 
Assess post-surgery 
Assess response to treatment 
Follow up recurrence and progression.
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17
Q

What are the management options for CNS tumours (3)

A

Surgery
Radiotherapy
Chemotherapy

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18
Q

What is the purpose of surgery for CNS tumours

A

Maximal safe resection aims to obtain an extensive excision with minimal damage to the patient

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19
Q

What determines the possibility of surgery for CNS tumours (3)

A

Age and performance status
Resectability: location, size, number of lesions
Histology (intraoperative diagnosis)

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20
Q

What are the types of radiotherapy available for CNS tumours (3)

A

Fractionated RT
Stereotactic radiosurgery
Whole brain

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21
Q

What are the indications for chemotherapy for CNS tumours

A

Mainly for high-grade gliomas (temozolomide)

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22
Q

What are the three main forms of neurosurgery (3)

A

Stereotactic biopsy - inoperable tumours (about 0.5cm tissue) - tissue may not be representative.
Open biopsy - inoperable but approachable tumours (about 1cm) - more accurate
Craniotomy for debulking (as much tissue as possible)

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23
Q

What is involved in the WHO classification of CNS tumours (2016) (4)

A

Tumour type - putative cell of origin
Tumour differentiation - grading
Tumour defined by genetic profile - integrated diagnosis

NO STAGING

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24
Q

What is the only CNS tumour that is staged

A

Medulloblastoma

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25
Q

How are tumours named (3)

A

One of three ways:
Descriptive - defined by histology
Names derives from putative cell of origin
Histological type

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26
Q

What is the point of grading a tumour

A

The grade is an attempt to stratify tumours by outcome - degree of malignancy

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27
Q

What is grading based on (2)

A 
Histopathological criteria (proliferative activity, differentiation, necrosis) 
Based on natural history
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28
Q

How many grades does WHO define for CNS tumours

A

4

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29
Q

What are the grades for CNS tumours (4)

A

Grade 1 - long-term survival/cured
Grade 2 - cause death in more than 5 years
Grade 3 - cause death within 5 years
Grade 4 - cause death within 1 year

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30
Q

What is more accurate: tumour typing or tumour grading

A

Tumour typing

Grading is less accurate than typing - limited by size of biopsies

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31
Q

What is the most common primary CNS tumour

A

Glial

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32
Q

What are the most common adult glial tumours (2)

A

Astrocytoma (grades 2-4)

Oligodendroglioma (grades 2-3)

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33
Q

What is the most common glial CNS tumour presentation in adults

A

Diffuse infiltration (Grades >2)

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34
Q

What are the most common childhood glial tumours (4)

A

Pilocytic astrocytoma (grade 1)
Pleomorphic xantoastrocytoma
Subependymal astrocytoma
Ganglioglioma

35
Q

What grades do childhood gliomas tend to be

A

Grades 1/2

36
Q

What is the most common glial CNS tumour presentation in children

A

Compressive margins - rare malignant transformation

37
Q

What mutation is present in diffuse gliomas

A

IDH mutations

38
Q

What mutation is present in circumscribed gliomas

A

MAPK pathway mutations

39
Q

What grade is a pilocytic astrocytoma

A

Grade 1

40
Q

When do pilycytic astrocytomas tend to occur

A

Usually 1st and 2nd decade

20% of CNS tumours are below age 14

41
Q

Where do pilocytic astrocytomas tend to occur (3)

A

Cerebellar
Optic-hypothalamic
Brain stem

42
Q

How do pilocytic astrocytomas appear on MRI

A

Always contrast enhancement and circumscribed (never diffuse infiltration)

43
Q

What is the hallmark of pilocytic astrocytoma (3)

A

Piliod ‘hairy’ cell
Very often Rosenthal fibres and granular bodies.
Slow growing: low mitotic activity

44
Q

What mutation is associated with pilocytic astrocytomas

A

BRAF

45
Q

When do diffuse astrocytomas tend to occur

A

Occur more commonly between 30-40 years

46
Q

What grade are diffuse astrocytomas

A

Grade 2

47
Q

How do diffuse astrocytomas appear on MRI/MRISpec (2)

A

`T1 hypointense, T2 hyperintense, non-enhancing lesion.

Low choline/creatinine ratio at MRSpec

48
Q

What are the histological features of diffuse astrocytoma (3)

A

Low to moderate cellularity
Mitotic activity is negligible or absent
Vscular proliferation and necrosis are absent

49
Q

What are the most common mutations in diffuse astrocytomas

A

IDH1/2

50
Q

What grade is a glioblastoma multiforme

A

Grade 4

51
Q

What age group are affected by glioblastoma multiforme

A

Most patients >50 years

52
Q

What is the appearance of glioblastoma multiforme on MRI

A

T1 enhancing post-contrast

53
Q

What are the histological features of glioblastoma multiforme (2)

A

High cellularity and high mitotic activity

Endothelial proliferation and/or necrosis

54
Q

What is the natural history of glioblastoma multiforme (2)

A

90% are de novo GBM (IDH wildtype)

10% are secondary GBM (progression - IDH mutation)

55
Q

What tumour can GBM progress from

A

Lower grade astrocytoma

56
Q

What are two characteristics of large tumours (2)

A

Neoangiogenesis - new blood cell formation

Central necrosis.

57
Q

What proportion of all brain tumours are oligodendrogliomas

A

5% of all primary CNS tumours

58
Q

What grade are oligodendrogliomas

A

Grade 2-3

59
Q

What age group do oligodendrogliomas affect

A

20-40 years

60
Q

How do oligodendrogliomas present

A

With a long history of neurological signs (e.g. seizures)

61
Q

What is seen on MRI of a oligodendroglioma

A

No or patchy contrast enhancement.

MRI and MRSpec are not predictive of transformation

62
Q

What are the histological features of oligodendrogliomas

A

Round cells with clear cytoplasm (friend eggs)

63
Q

What mutation is associated with oligodendroglioma

A

IDH1/2 + codeletion 1p/19q

64
Q

What is the prognosis for oligodendroglioma (3)

A

Better prognosis than astrocytomas
Slow growth - resection is important
Better response to chem and radiotherapy.

65
Q

What grade are meningiomas

A

Grade 1-3

80% Grade 1: benign, recurrence <25%
20% Grade 2: atypical, recurrence 25-50%
1% Grade 3: malignant, recurrence 50-90%

66
Q

What proportion of primary CNS tumours are meningiomas

A

Second most common after gliomas.
25-30% of primary intracranial tumours
10% incidental in post-mortem

67
Q

What age group are most affected by meningiomas

A

> 40 years

68
Q

Where do meningiomas tend to occur

A

Any site of craniospinal axis

69
Q

What are the symptoms of meningiomas

A

Focal signs (seizures, compression)

70
Q

What is seen on MRI of a meningioma (3)

A

Extraaxial
Isodense
Contrast-enhancing

71
Q

What is the most important factor in meningioma diagnosis and treatmetn

A

Most meningiomas are benign and slow-growing, so histology is crucial

72
Q

What determines tumour grade (meningiomas)

A

Mitotic activity

Mitoses/10HPF: <4 = grade 1; 4-20 = grade 2; >20 = grade 3

73
Q

What is crucial to determine (other than grade) in meningiomas

A

Brain invasion.
Interface between tumour and the cortex should be carefully examined.
Pseudoinvasion along the virchows-Robin space is possible

74
Q

What grade are medulloblastomas

A

Grade 4

75
Q

Where do medulloblastomas originate

A

Embryonal tumour - originate from neuroepithelial precursurs of the cerebellum/dorsal brianstem

76
Q

What is the prevalence of medulloblastoma

A

It is a rare tumour (2/1,000,000/year), but it is the second most common brain tumour in children

77
Q

What are the histological features of medulloblastoma

A

Small blue round cell tumour

78
Q

What are the molecular classifications of medulloblastoma (3)

A

WNT-activated
SHH-activated
non-WNT/non-SHH

79
Q

What radically improves outcome in medulloblastoma

A

Radio-chemotherapy

80
Q

Where do medulloblastoma tend ot occur (2)

A

In children - vermis

Adults - hemispheric

81
Q

What is the most frequent CNS tumour

A

CNS metastases

82
Q

What are the most common tumours that give rise to CNS metastases (5)

A 
Lung cancer 
Melanoma 
Breast cancer 
Renal cancer 
Colon cancer
83
Q

What is the prognosis for brain metastases

A

Very poor

Kat's Pathology (105 decks)

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