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FRCR2 > CNS > Flashcards

CNS Flashcards

1
Q

When is surgery considered as treatment for intracranial mets

A

Solitary or large met (>3cm)
Mets causing obstruction - hydrocephalus or raised ICP
Cystic or necrotic mets where SRS is less likely to be effective
Where histology / diagnosis will be helpful

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2
Q

What are the criteria for SRS

A

An established diagnosis of cancer
Karnofsky performance status (KPS) >70
Absent or controllable primary disease (staging scans within last 3 months)
No previous SRS/SRT in the last 3 months
Life expectancy from extracranial disease >6 months
No pressure symptoms best relieved by surgery
Contrast enhanced MRI to evaluate cerebral metastases
Maximum combined treatment volume <20cc
Discussion at an MDT meeting

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3
Q

What are the histological signs of a GBM

A

Epithelial / vascular proliferation
Necrosis

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4
Q

What is the pathognomic mutation of an oligodendroglioma

A

1p19q co-deletion

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5
Q

What is the pathognomic mutation of an astrocytoma (presuming high grade ancestry)

A

ATRX loss
(ATRX retained = GBM)

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6
Q

What is bright on a T1 MRI

A

T1 -> CSF dark & Tumour dark

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7
Q

What is bright on T2 MRI & FLAIR

A

T2 -> CSF white & Tumour bright
T2 FLAIR -> CSF dark & Tumour bright with Oedema

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8
Q

How do low & high grade tumours enhance with contrast

A

Low grade tumours tend not to enhance. High grade tumours enhance (due to endothelial / vascular proliferation)

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9
Q

Where and for whom do pilocytic astrocytomas occur, and how do they present

A

Typically in children, low grade tumour
Tend to occur in cerebellum, causing obstruction and obstructive symptoms (pressure sx and headache)

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10
Q

What is the dose fractionation for craniospinal irradiation for a non-germinoma

A

overall 54Gy/30#
36Gy/20# to whole CSA and 18Gy/10# boost to tumour bed

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11
Q

What are the most common mutations seen in a pleomorphic xanthoastrocytoma

A

CDKN2A/B deletions (>90 percent)
BRAF V600E mutations (60-80 percent)

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12
Q

What is the benefit of adjuvant PCV in grade 2 gliomas

A

Buckner - 2016 NEJM
high risk Gr2 gliomas (<40yrs and subtotal resection or >40yrs) randomised to 6x adj PCV or not
PCV increased mOS to 13.3yrs from 7.8, and 10yr OS from 40% to 60%

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13
Q

When is immediate post-op RT indicated for a grade 2 glioma, vs at progression?

A

2 of:
Age >40
>6cm
Enhancement
Astrocytic
Crossing midline
Symptomatic

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14
Q

What is the adjuvant RT dose for a low grade glioma

A

54Gy/30#

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15
Q

What is the adjuvant dose for a grade 3 anaplastic oligodendroglioma

What adjuvant chemotherapy regime would typically follow

A

59.4/33#

Followed by 6x PCV adjuvant chemotherapy

12mth OS benefit to PCV adjuvantly

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16
Q

What is the adjuvant dose for a grade 3 anaplastic astrocytoma

What adjuvant chemotherapy regime would typically follow

A

59.4Gy/33#

Temozolamide x12 cycles adjuvantly
Based on the catnon trial

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17
Q

What is the post-operative RT regimen for a Gr4 GBM if <70 and PS0-1

And if <70 & PS2

And if >70

A

Concomitant CRT
60Gy/30# + concurrent TMZ (75mg/m2) & Co-trimoxazole prophylaxis 480mg M/W/F

Adjuvant chemotherapy
Adjuvant TMZ 150 - 200mg/m2 - D1-5 Q28 for 6 cycles

If <70 but PS2 - give RT only, without temozolamide

> 70: 40Gy/15# +/- concomitant tmz, and based on methylation status (based on Perry trial)

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18
Q

What RT dose is given to a butterfly glioma

A

30Gy/6# over 2wks, treating M/W/F

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19
Q

What is the mOS for a GBM? and based on what trial

A

Stupp trial
CRT & adj tmz vs RT alone
Median OS: 14.6m vs 12m and PFS 6.9m vs 5m
2yr OS: 26.5% vs 10.4%

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20
Q

Diffuse midline H3 K27-altered midline gliomas area considered what grade

Where are they typically located

How do they typically present

A

Always grade 4

most commonly in the pons, or other midline structures
(thalamus, brainstem - mesencephalon, pons, medulla, spinal cord)

Present with CN palsies, raised ICP and cerebellar sx.

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21
Q

What is the dose objective & constraint to brainstem

A

54Gy (objective)
59Gy (constraint)

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22
Q

What is the dose constraint to spinal cord

A

48Gy (objective)
50Gy (constraint)

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23
Q

What is the dose objective & constraint to optic nerves

A

50Gy (objective)
54Gy (constraint)

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24
Q

What is the dose objective & constraint to optic chiasm

A

50Gy - objective
54Gy - constraint

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25
Q

What is the dose objective & constraint to lens

A

6Gy objective
10Gy constraint

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26
Q

What is the dose objective & constraint to globes

A

O - 40Gy
C - 45Gy

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27
Q

What is the dose constraint to cornea

A

30Gy

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28
Q

What is the dose objective & constraint to retina

A

O - 45Gy
C - 50Gy

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29
Q

What is the dose constraint to lacrimal gland

A

Mean <26Gy

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30
Q

What is the dose constraint to cochlea

A

Mean <45Gy

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31
Q

What are the acute side effects of brain RT

A

Alopecia
Headache
Nausea/vomiting

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32
Q

What are the late side effects of brain RT

A

Neurocognitive effect – memory loss
Pituitary hypofunction
Optic chiasm damage – may cause blindness
Cataracts
2nd malignancies = 1%

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33
Q

What is the most common malignant tumour of the eye and ocular adenexa.

A

Non-hodgkin lymphoma - treat as primary CNS lymphoma

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34
Q

Where do ependymomas originate from
Where is the commonest site

How do they present

A

Ventricles - can occur anywhere within the neuraxis, and most commonly in the spine.
Most common intracranial site is the posterior fossa

Commonly cause obstruction, so tend to present with hydrocephalus

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35
Q

How are ependymomas graded

A

G1 - subependymomas or myxopapillary ependymomas
G2 - cellular, papillary, clear cell and tanycytic
G3 - anaplastic

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36
Q

What are the investigations for an ependymoma

A

MRI brain and whole spine
MRI post op for extent of resection
CSF 14 days post op to exclude circulating tumour cells

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37
Q

How is an ependymoma treated, and what determines adjuvant tx

A

surgery - resection of primary and mets

adjuvant tx if incompletely resected

if completely resected:
G1 - surveillance only
Gr2 - consider adjuvant tx
Gr3 - RT (adults = involved field only, children = whole CSA)

disseminated - CSRT with boost to all disease sites

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38
Q

How is the radio sensitivity of germinomas

Where in the CNS do they tend to occur

A

very radiosensitive

midline - pineal gland, suprasellar

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39
Q

What ix are required prior to starting treatment for a germ cell tumour

A

Ophthalmological assessment
Audiogram
GFR for patients due chemotherapy
Endocrine assessment as indicated
Fertility preservation

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40
Q

How is the management of germ cell tumour categorised?

A

Non-secreting germ cell tumour
NS-GCT - 80% secreting

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41
Q

For a non-secreting germ cell tumour, how is treatment divided

A

Localised
Metastatic

42
Q

What is the treatment for a non-secreting localised germ cell tumour

A

Localised - 4x PIE chemotherapy (cisplatin, ifosfamide, etoposide), followed by CSRT
40Gy/25#overall, 24Gy/15# to CSA with 16Gy/10# boost to primary site

43
Q

What is the treatment for a non-secreting metastatic germ cell tumour

A

No chemo
24Gy/15# CSA + tumour site boosts

44
Q

For a secreting germ cell tumour, how is treatment divided

A

Localised
Metastatic

45
Q

What is the treatment for a secreting localised germ cell tumour

A

4 x PIE (neo-adjuvant)
RT - 54Gy in 30# to primary (consider surgery for residual disease)

46
Q

What is the treatment for a secreting metastatic germ cell tumour

A

Phase 1 CSA - 30Gy in 20#
Phase 2 boost - brain sites - 24Gy in 15#; spinal sites - 16Gy in 10#

47
Q

Where do medulloblastomas tend to occur
How do they tend to present

A

Posterior fossa - midline for children, more likely lateralised for adults

Tend to present with symptoms of obstruction / raised ICP, or cerebellar symptoms

48
Q

What are the four subtypes of medulloblastoma

A

WNT, SHH, Group 3, Group 4

49
Q

How are medulloblastomas investigated / worked up

A

1/3 metastasise through the CNS, so pts need a spinal MRI and LP pre-operatively, or 14 days post op, to exclude circulating tumour cells

50
Q

What investigations are done post-op

A

MRI for possible residual disease
If resectable, further surgeries are done for the most complete resection possible

51
Q

How are medulloblastomas pts categorised post operatively

A

Standard or high risk

Standard risk
>3yrs age
<1.5 cm residual disease
CSF cytology negative
M0 (no metastases) on spinal staging
WNT and SHH groups

High risk
>1.5cm residual disease
CSF cytology positive (LP)
Metastases on staging imaging
Group 3 or 4 histology

52
Q

What adjuvant treatment is given for medulloblastoma

A

CSA RT followed by chemotherapy

53
Q

How is the CSA dose for medulloblastoma split

What adjuvant treatment is given following RT

A

High risk: 36Gy/20# to whole CSA followed by 18Gy/10# boost to primary site, with vincristine

Standard risk: 23.4Gy/13#, followed by primary site boost of 30.6Gy/17#, with vincristine

Overall primary tumour receives 54Gy/30#, with standard risk receiving less to the CSA

Following RT, adjuvant chemo is given
High risk - 8x PACKER chemotherapy
Lomustine, cisplatin, vincristine

Standard risk - modified PNET5 regime
Alternating cycles x8 of platinum/vincristine/lomustine, and cyclophosphamide

54
Q

What is the prognosis of treated medulloblastoma

A

5YS approx 80%
10YS 60%

55
Q

When is CSA RT indicated, and to what dose

A

All primitive neuroectodermal tumours (PNET):
Medulloblastoma - 54Gy/30# overall, according to risk
Supratentorial PNET
Pineoblastoma

Germ cell tumours:
Germinoma

Disseminated pilocytic astrocytoma

Ependymoma:
Gr3 adjuvantly for limited disease, or disseminated

56
Q

What is the RT dose to the pituitary
What is the CTV, and margins

A

Primary RT: 45Gy/25# or 54Gy/30# if functioning

Adjuvant RT for macroscopic residual disease: 50.4Gy/28#

GTV = residual disease, and reconstructed pre-op disease
CTV = GTV +5mm, and whole pituitary fossa
PTV = CTV +3mm

57
Q

How do low grade gliomas appear on MRI

A

T1 - typically hypo intense and do not take up contrast
T2 - Tumour is hyperintense, but no oedema
Hyperintense on FLAIR

58
Q

How do high grade gliomas appear on MRI

A

Hyper-intense on T1, take up contrast

59
Q

How does a pilocytic astrocytoma typically present

A

Most commonly occurs in children/TYA, and cerebellum, presenting with obstructive sx

60
Q

How is a pilocytic astrocytoma treated

A

Localised
Tx: Surgery

Disseminated
Rare, usually arise in cerebellum, hypothalamic region, optic chiasm, near brainstem

Tx: Craniospinal RT
To start within 4 weeks of diagnosis
Ph1 - CSRT - 24Gy/12# over 4 weeks (1.8Gy/#)
Brain boost to tumour bed - 18Gy in 10# over 2 weeks
+/- SC boost to disease sites - 14.4Gy in 10# over 2 weeks

GTV - All visible enhancing tumour on T1W + gad
CTV - GTV + 5mm, and include all meningeal reflections throughout CSA
PTV - CTV + 5mm (but CTV-PTV margin depends on site within CSA - larger margins lower down the spine)

61
Q

When should temozolamide and co-trimoxazole be held for deranged LFTs

A

ALT >148, ALP >325, bili >32 - hold co-trimox and tmp, until LFTs improved
ALT >245 / ALP >650 / Bilirubin >63 - discontinue both and continue RT alone

62
Q

How does a craniopharyngioma typically present

A

Visual defect: lower quadrantanopia (tumour pushes down from above)
Hormone deficiency & Raised prolactin due to compression of pituitary stalk
Headaches
Obstructive hydrocephalus
Diabetes insipidus - loss of ADH

63
Q

How is a craniopharyngioma treated

A

Surgery if possible
Primary RT if inoperable , or incomplete resection / recurrence.

RT 75-90% effective for local control

Dose:
Adjuvant - 50Gy/30#
Primary dose - 54Gy/30#

GTV-CTV: 5mm
Need to include all areas contacted by the tumour in the CTV

CTV-PTV: 3mm”

64
Q

When is RT indicated for an ependymoma

And at what dose

How is metastatic ependymoma treated

A

Indications:
Incomplete resection if pt >3 yrs old.
G3 tumours or high risk grade 2
Relapsed disease if no previous RT

Adjuvant:
Intracranial - 54Gy in 30# (protons for children)
Spinal cord - 50-55Gy in 30-33#
Cauda equina - 54Gy in 30# - 59.4Gy in 33#

Metastatic:
CSRT -> 36Gy in 20# to CSA & Boost to individual sites of spinal disease up to 50.4Gy

If there is positive cytology and no gross visible disease outside the primary location, give craniospinal fields 45Gy/25# over 5 weeks followed by boost of 14.4Gy/8#”

65
Q

What is the grading of ependymoma

A

G1-3

66
Q

What proportion of pituitary macro adenomas are non-secreting

A

25%

67
Q

What CNs are affected for a cavernous sinus tumour

A

III, IV, Va, Vb, VI

68
Q

What are the treatment options for a pituitary tumour

A

If non-secreting and no threat to vision - observation

If secretary and threat to vision:
Medical - cabergoline/bromocriptin/lanreotide
Surgical - indicated if threat to vision or failure of medical mx
Surgical approach is typically trans-sphenoidal, but can be transcranial if there is superior extension

RT: failure of medical mx but not fit for surgery, recurrence or residual disease, or persistent hormone secretion

69
Q

What is the outcome of surgery + RT to a pituitary tumour

A

80% relapse free survival at 20 years

70
Q

What is the rate of optic neuropathy for pituitary RT

A

1%

71
Q

How would a VS typically present

A

Unilateral sensorineural hearing loss
Balance problems
Involvement of the trigeminal nerve - facial pain or altered sensation

May expand into posterior fossa to occupy cerebellopontine angle -> compression of CN V, VII, VIII

72
Q

What is the treatment for a VS

A

Only 50% grow - monitor for serial growth
Surgery for young pts and large / cystic tumours
RT - SRS 12Gy/1# or 50Gy/30# fractionated

73
Q

What margin is used for VS SRS & fractionated

A

SRS:
GTV-CTV = 0mm
CTV - PTV=1mm

Fractionated:
GTV = CTV = visible tumour
CTV-PTV = 3mm

74
Q

where are the typical sites of chordoma
What marker is sent
What is the complication

A

Spheno-occipital
Sacral spine

Brachyury

Chordomas tend not to metastasise but can be locally invasive and have a high rate of recurrence

75
Q

What is the management of a skull base chordoma

A

Surgery and adjuvant RT, typically protons given location at the skull base
Chordomas receive 70Gy, chondrosarcomas 72Gy

CTV:
Clival lesion - GTV + entire clivus / sphenoid bone
Vertebral lesion - GTV + entire vertebral body

PTV:
CTV +3mm for skull base, 7mm elsewhere

76
Q

Where do paragangliomas tend to occur

What investigation is needed before treatment

What mutation is associated

A

Paravertebral - tend to be chromatin positive and release catecholamines
In relation to the great vessels of the head and neck eg carotid bodies - tend to be chromatin negative and don’t release catecholamines

Need MRI brain and whole spine to exclude phaeochromocytoma

10% are associated with SDH-mutation

77
Q

What is a glomus jugulare tumour

A

Paraganglioma arising from jugular foramen
Affect CN IX, X and XI

78
Q

How are meningiomas classified

A

Gr1-3

79
Q

How is a meningioma resection classified

A

Simpson classification

Stage 1 - Complete excision including dura and bone (tumour + dura + bone)
Stage 2 - Complete excision with co-angulation of dural attachment (tumour + dura)
Stage 3 - Excision of intradural tumour, without resection of it’s dural attachment (tumour alone)
Stage 4 - Partial removal, leaving intradural tumour in-situ
Stage 5 - Decompression only +/- biopsy

80
Q

What are the indications for RT for a meningioma

A

Primary RT:
Option for Grade 1
Inoperable
Recurrence

Adjuvant RT:
Incomplete resection
Grade 3
Atypical histology

81
Q

What RT dose is given to meningioma

A

SRS - 12-15Gy/1#

Fractionated:
G1 - 50Gy/30#
G2 - 55Gy/33# if Grade 2
G3 or sarcomatoid - 60Gy in 30#

82
Q

What dose is given to an optic nerve sheath meningioma

A

54Gy in 30#

83
Q

What are the RT volumes for a meningioma

A

Residual disease
GTV - Residual meningioma, hyperostotic bone and dural extension
CTV - GTV +1cm in plane of dura
If brain invasion present -> add 1cm into brain from brain/meningioma margin
Include all abnormal, hyperostotic bone not in GTV
PTV - CTV + 3mm

No residual disease
GTV - Pre-op MRI -> define largest extent of dural/bone thickening
CTV - GTV + 1cm in plane of dura, if documented brain invasion add 1cm into brain
Include all abnormal, hyperostotic bone
PTV - CTV + 3mm

84
Q

What is the treatment algorithm for a low grade glioma

A

Maximal surgery +/- RT (50.4Gy/30#) & 6x PCV

85
Q

What are the indications for upfront RT & ChT for a low grade glioma

A

Large initial tumour, >4cm
Incomplete resection / residual disease
>40yrs
New or worsening neurological deficit

86
Q

Does vincristine need dose adjustment for renal or hepatic impairment

A

No renal dose adjustment
Adjusted for hepatic impairment if bili >51 or ALT/AST >60

87
Q

What is the prognosis of a Gr3 oligo

A

5-yr OS: 30-35%
mOS 42mths with RT and PCV (oligo)

88
Q

What is the prognosis of a Gr3 astro IDH mut

A

60-100mths

89
Q

What is the prognosis of a Gr4 astro IDH mut

A

36mths

90
Q

What is the prognosis of a GBM IDH WT, mgmt methylated

A

20mths

91
Q

What is the prognosis of a GBM IDH WT, mgmt unmethylated

A

12mths

92
Q

What is the prognosis of a Gr1 tumour

A

Curable
80% 10yr survival

93
Q

What is the prognosis of a Gr2 tumour

A

med survival 5-10yrs
Buckner - mOS 13.3yrs with RT & PCV

94
Q

What is the prognosis of a Gr4 tumour

A

Stupp trial (age <70yrs) - mOS 14.6 for GBM with tx
2yr OS 26%
Pts with complete resection did better, and methylated MGMT vs unmethylated

95
Q

What is the prognosis of a GBM if age >70

A

Perry 2017 - 9.3mths with treatment (up to 12 cycles adj tmz) for all comers
If methylated - mOS 13.5mths
Most benefit in those with MGMT methylation. No statistically significant benefit for tmz if unmethylated

96
Q

How is a pineoblastoma treated

A

NA vinc/carbo/etopo x3-4 (treat like neuroendocrine)
CSA RT

97
Q

what factors should be considered when considering RT treatment of brain mets

A

Controlled or treatable extracranial disease
PS 0-1 (KPS ≥70)
Prognosis of at least 6mths

98
Q

When should fractionated intracranial RT be considered over SRS
What dose is typically used for fractionated RT

A

Lesions larger 2-3cm, lesions close to a critical OAR or where V12Gy ≥10cm3 (the volume of normal tissue, excluding GTV, that receives at least 12Gy)

V12Gy of 5, 10 and >15cm3 = risk of radio necrosis of 10%, 15% and 20%

27Gy/3# or 30Gy/5#

99
Q

What is the commonest primary brain tumour in adults

A

meningiomas, followed closely by astrocytomas

100
Q

What is the commonest malignant brain tumour in adults

A

glioblastoma multiforme.

101
Q

What is the commonest brain tumour and malignant brain tumour in children

A

low grade pilocytic astrocytomas
commonest malignant primary brain tumours -medulloblastomas

FRCR2 (38 decks)

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