Intracranial Tumours Flashcards

1
Q

What are the most common CNS tumours?

A
  • Gliomas

- Meningiomas

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2
Q

What structures are involved in gliomas?

A

Glia cells.

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3
Q

What structure is involved in mimningiomas?

A

Meninges.

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4
Q

Who is gliomas most common in?

A

Males.

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5
Q

Who is meningiomas most common in?

A

Females.

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6
Q

What CNS tumours are common in children?

A
  • Cerebellum
  • PNET (primitive neuroectodermal tumour)
  • Ependymoma
  • Germ cell
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7
Q

Why don’t neurons give rise to many tumours?

A

Not in direct contact with the blood supply.

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8
Q

What connects neurons to the blood supply?

A

Glia cells.

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9
Q

What are the 4 types of glia cells?

A
  • Astrocytes
  • Oligodendrocytes
  • Ependymal cells & choroid plexus cells
  • Microglia
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10
Q

What is the function of astrocytes?

A

Support and protection.

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11
Q

What is the function of oligodendrocytes?

A

Myelinating cells.

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12
Q

What is the function of ependymal cells & choroid plexus cells?

A

Produce CSF.

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13
Q

What is the function of microglia?

A

Defence (resident macrophages in CNS).

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14
Q

What are the 5 main CNS tumours?

A
  • GLIOMA (glia cells)
  • MENINGIOMA (meninges)
  • NEUROCYTOMA (neuronal)
  • PNET (primitive)
  • SCHWANNOMA (nerve sheath)
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15
Q

What are the 3 types of glioma?

A
  • Astrocytoma
  • Oligodendroglioma
  • Ependymoma
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16
Q

How are tumours differentiated histologically?

A

Name of the tumour.

-e.g. glioblastoma

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17
Q

How are tumours differentiated based on biological behaviour?

A

Grade of the tumour (how malignant).

-I to IV

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18
Q

What is the biological behaviour of a benign tumour? (4)

A
  • slow growing
  • no/slow progression
  • respect surrounding tissue
  • no recurrence
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19
Q

How are benign tumours treated?

A

Surgery.

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20
Q

What is the biological behaviour of a malignant tumour? (4)

A
  • fast growing
  • progress
  • invade surrounding tissue
  • recurrent
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21
Q

How are malignant tumours generally treated?

A

Surgery and adjuvant therapy.

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22
Q

Why can benign tumours in the thalamus/brainstem be fatal?

A

Almost impossible to remove.

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23
Q

How are tumours graded?

A

I to IV.

-I = most mild, IV = most severe

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24
Q

What are the characteristics of a grade I tumour?

A
  • Benign
  • No recurrence
  • No/slow porgression
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25
Q

What are the characteristics of a grade II tumour?

A
  • Low grade
  • Some progression
  • Astrocytoma (6-7 yr life expectancy)
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26
Q

What are the characteristics of a grade III tumour?

A
  • High grade
  • Rapid progession
  • Astrocytoma (2-3 yr life expectancy)
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27
Q

What are the characteristics of a grade IV tumour?

A

-Very agressive

-

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28
Q

What is the histological criterai for a malignancy in brain tumours? (4)

A
  • No stability (cell density & atypia)
  • More mitotic activity
  • Necrosis (compete for resources)
  • Vascular proliferation
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29
Q

What are the microscopic features of astrocytic tumours?

A
  • Microcystic background
  • Increased cell density
  • Pleomorphism (size/shape)
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30
Q

What are the main features of a diffuse astrocytoma? (4)

A
  • Infiltrative & microcystic
  • Fibrillary
  • Low cellular density
  • Mild atypia
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31
Q

What grade are diffuse astrocytomas normally?

A

Grade II.

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32
Q

Do diffuse astrocytomas have mitotic activity?

A

No.

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33
Q

What is the median survival for diffuse astrocytomas?

A

80 months.

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34
Q

What are the main features of anaplastic astrocytomas? (2)

A
  • Moderate cellular density

- Moderate pleomorphism

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35
Q

Do anaplastic astrocytomas have mitotic activity?

A

Yes.

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36
Q

What grade are anaplastic astrocytomas normally?

A

Grade III.

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37
Q

What is the median survival for anaplastic astrocytomas?

A

30 months.

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38
Q

What are the main features of glioblastomas? (3)

A
  • High cellular density
  • Necrosis
  • Vascular proliferation
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39
Q

What grade are glioblastomas normally?

A

Grade IV.

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40
Q

Do glioblastomas have mitotic activity?

A

Yes - lots.

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41
Q

What is the median survival time for glioblastomas?

A

10 months.

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42
Q

What are the main features of pilocytic astrocytomas? (6)

A
  • Children
  • Cerebellum
  • Well defined
  • Vascular proliferation
  • Cystic
  • Rosenthal fibres
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43
Q

What grade are pilocytic astrocytomas normally?

A

Grade I.

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44
Q

What are the main features of oligodendrogliomas? (3)

A
  • Round nucleus with clear cytoplasm (FRIED EGG)
  • Arborising capillaries
  • Calcifications
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45
Q

What grade are oligodendrogliomas normally?

A

Grade II-III.

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46
Q

What are the main features of ependymomas? (4)

A
  • Round uniform cells
  • Well-defined tumour
  • Pseudorosettes
  • Ventricles
47
Q

What grade are ependymomas normally?

A

Grade II-III.

48
Q

What are the main features of meningiomas? (6)

A
  • Females
  • Dura
  • Well-defined
  • Extra-axial tumours
  • Whorls
  • Psammoma bodies
49
Q

What grade are meningiomas normally?

A

Grade I.

50
Q

What are the main features of PNET - medulloblastomas? (5)

A
  • Children
  • Cerebellum
  • Very high cellular density
  • Anaplastic hyperchromatic cells
  • Rosette
51
Q

What grade are PNET - medulloblastomas normally?

A

Grade IV.

52
Q

Do PNET - medulloblastomas have mitotic activity?

A

Yes, frequent.

53
Q

Which tumours mainly affect the cerebellum in children? (2)

A
  • PNET - medullblastomas

- Policytic astrocytomas

54
Q

Which tumours mainly affect females?

A

Meningiomas.

55
Q

Which tumours mainly affect males?

A

Gliomas.

56
Q

What type of tumour has a median survival rate of 10 months?

A

Glioblastoma.

57
Q

What type of tumour cell has a ‘fried egg’ appearance (round nucleus, clear cytoplasm)?

A

Oligodendroglioma.

58
Q

What type of tumour contains rosenthal fibres?

A

Pilocytic astrocytoma.

59
Q

What is a rosenthal fibre?

A

A long, thick eosinophlic (pink) bundle seen in some slow growing astrocytomas.

60
Q

What type of tumour is extra-axial, and where is it found?

A

Meningioma - in dura.

NB. cannot cross BBB

61
Q

Name 3 types of nerve sheath tumours.

A
  • Spindle cell tumours
  • Schwannoma
  • Neurofibroma
62
Q

What grade are schwannomas and neurofibromas?

A

Both grade I.

63
Q

Why can schwannomas be problematic?

A

They are benign, but can be difficult to remove.

64
Q

What cranial nerve do schwannomas affect?

A

Cranial nerve VIII.

65
Q

Give 2 features of schwannomas.

A
  • BIPHASIC pattern (loose and dense areas)

- RETICULIN fibres (type III colagen)

66
Q

What structures do neurofibromas affect?

A

Spinal nerves.

67
Q

What substance are neurofibromas rich in?

A

Collagen.

68
Q

What do signalling pathways normally do?

A

Regulate self-renewal during stem cell development.

69
Q

What does the Wnt signalling pathway normally act on? (3)

A
  • Haemopoietic
  • Epidermal
  • Gut
70
Q

What tumours form if there is a mutation in the Wnt signalling pathway? (2)

A
  • Colon carcinoma

- Epidermal tumours

71
Q

What does the Shh signalling pathway normally act on? (3)

A
  • Haemopoietic
  • Neural
  • Germ line

NB same as Notch

72
Q

What tumours form if there is a mutation in the Shh signalling pathway?

A
  • Medulloblastoma

- Basal cell carcinoma

73
Q

What does the Notch signalling pathway normally act on? (3)

A
  • Haemopoietic
  • Neural
  • Germ line

NB same as Shh

74
Q

What tumours form if there is a mutation in the Notch signalling pathway?

A
  • Leukaemia

- Mammary tumours

75
Q

How do astrocytes lead to glioblastoma formation?

A

Astrocyte
»astrocytoma
»anaplastic astrocytoma
»glioblastoma (2*)

76
Q

Name 2 possible events that occur to transform astrocytes into astrocytomas.

A
  • Chromosome 17p loss

- TP53 gene mutation

77
Q

What happens to cause astrocytomas to transform into anaplastic astrocytomas?

A

Chromosome 19q loss.

78
Q

Name 2 possible events that occur to transform anaplastic astrocytomas into glioblastomas.

A
  • Chromosome 10q loss

- Hypermethylation of RBI gene

79
Q

What are the 4 markers that are most relevant for molecular diagnostics of gliomas?

A
  • MGMT promoter methylation
  • 1p/10q deletion
  • IDH1/IDH2 mutation
  • BRAF duplication/fusion
80
Q

What type of tumour is only affected by MGMT methylation?

A

Primary glioblastoma.

Grade IV

81
Q

What type of tumour is only affected by BRAF alteration?

A

Pliocytic astrocytoma.

Grade I

82
Q

How do intracranial tumours often present? (5)

A
  • ^ Intracranial pressure
  • Epilepsy
  • Neurological deficit
  • Endocrine dysfunction
  • Focal symptoms (e.g. weakness)
83
Q

What are symptoms of raised intracranial pressure? (3)

A
  • Headaches (morning)
  • Vomiting
  • Blurred vision
84
Q

What may cause a raised intracranial pressure? (3)

A
  • Tumour
  • Oedema
  • Obstructive hydrocephalus
85
Q

What type of tumour may cause seizures?

A

Supratentorial.

86
Q

What are the 5 main types of neurological deficits?

A
  • Cognitive
  • Visual
  • Cranial nerve
  • Motor
  • Sensory
87
Q

What may endocrine dysfunction lead to?

A
  • Over-production

- Under-production

88
Q

What do haematological investigations often show with intracranial tumours?

A

Increased haemoglobin.

89
Q

What tumour markers are tested for? (3)

A
  • aFP
  • Bhcg
  • PSA
90
Q

Why are chest x-rays often carried out with suspected intracranial tumours?

A

To detect 2* tumours.

91
Q

How are intracranial tumours managed? (3)

A
  • MEDICAL (steroid/anti-convulsants/hormone replacement)
  • SURGICAL
  • ADJUVANT THERAPY (chemo-/radio- therapy)
92
Q

What are the 2 surgical options for intracranial tumours?

A
  • Biopsy

- Excision (total/partial)

93
Q

What is 5-ALA and how is it used for surgery?

A

A chemical given before surgery&raquo_space; highlights tumour cells.

94
Q

What are gliadel wafers?

A

Implants placed in space where tumour was during surgery&raquo_space; chemotherapy to the brain.

95
Q

What is stereotactic surgery?

A

Minimally invasive surgery - uses 3D systems to locate the target.

96
Q

Are meningiomas and schwannomas normally benign or malignant?

A

Benign.

97
Q

What are the main effects of steroid treatment?

A

Decreased swelling, but&raquo_space; weight gain.

98
Q

What are possible side effects of anti-epileptics?

A
  • Personality changes

- Drug interactions

99
Q

Which hemisphere is normally dominant?

A

Left hemisphere.

-i.e. concerned with language

100
Q

What is radiotherapy?

A

Use of x-rays to treat tumours.

-CT/MRI used to locate target first

101
Q

What is proton therapy?

A

Type of external beam radiotherapy.

-Doesn’t increase chance of cure, but lower doses of radiation&raquo_space; non-target regions

102
Q

Who is proton therapy predominantly used for?

A

Children.

103
Q

What are the acute side effects (days to weeks) of cranial radiotherapy? (3)

A
  • Cerebral oedema (» ^ICP)
  • Hair loss
  • Scalp/ear erythema
104
Q

What are the intermediate (weeks to months) side effects of cranial radiotherapy? (1)

A

Somnolence syndrome (severe tiredness).

105
Q

What are the late (months to years) effects of cranial radiotherapy?

A

Damage to sensitive structures&raquo_space; cataracts, hypothyroidism, memory loss.

106
Q

What are the median survival for the different grades of gliomas?

A

GRADE 1 : many years
GRADE 2 : 5-12 years
GRADE 3 : 2-4 years
GRADE 4 : 6-18 months

107
Q

Give 2 examples of CNS tumours that can be cured by treatment.

A
  • Germ cell tumours

- Medulloblastomas

108
Q

What are the 2 main purposes of chemotherapy?

A
  • Palliative treatment

- Enhance effectiveness of radiotherapy

109
Q

CASE 1 : fit and 2 week of gradual numbness and weakness on RHS.
-where is the lesion?

A

Left fronto-parietal region.

110
Q

What is the left fronto-parietal region often affected by?

A

Strokes.

-Middle cerebral artery

111
Q

CASE 2 : woman seems to understand what is said to her, but makes no sense. She is right handed.
-where is the lesion?

A

Left temporo-frontal region.

-Broca’s area

112
Q

What speech disorder is caused by damage to the Broca’s area?

A

Expressive aphasia.

113
Q

CASE 3 : 50 yr old, beomes withdrawn & bad-tempered, then develops headaches and vomiting.
-where is the lesion?

A

Frontal regions.

NB can tolerate big pathology before symptoms appear

114
Q

CASE 4 : 55 yr old, cannot dress herself properly, has numbness down one side and develops headaches.
-where is the lesion?

A

Parietal lobe.

|&raquo_space;sensory loss, dyspraxia and inattention