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H/O RC > CNS > Flashcards

CNS Flashcards

1
Q

medulloblastoma subgroups, epi, associated mutations, px

A

WNT (10%): CTNNB1, monosomy 6, DDX3X, APC
- best px, >90%

SHH (30%): PTCH1, PTCH2, SMO, SUFU, TP53
- 75% desmoplastic/nodular (TP53 WT) - OS 80%
- LC/A OS <50%

Group 3 (25%): i17q, MYCN amp (15%)
- worst px, 50-70% (worse with MYCN)

Group 4 (40%): i17q, MYCN amp, CDK6 amp
- 75%

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2
Q

medulloblastoma histo subgropus

A

classic
desmoplastic/nodular
extensive nodularity (MBEN)
large cell/anaplastic

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3
Q

Medulloblastoma staging

A

post op MR brain/spine
CSF 14d post op

M0: none
M1: CSF +
M2: gross nodular seeding subarachnoid
M3: gross nodular seeding spinal subarachnoid
M4: extraneural mets

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4
Q

Medulloblastoma risk stratification and associated px

A

Average: M0, <1.5 cm2 residual, undifferentiated histo, >3yo
- 5yDFS >80%

High
- 5yOS 40-6%
- <3yo except MBEN which ash excellent px

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5
Q

craniopharyngioma px factors

A

extent of resection (goal GTR)
younger age

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6
Q

craniopharyngioma tx

A

Controversial

maximal safe resection
- high perioperative M+M
STR -> RT

Omira reservoir (cyst drainage)
intracystic decompression and chemo (interferon alpha, bleo)
peglyated interferon alpha
BRAFi

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7
Q

CNS tumors dx without biopsy

A

DIPG: imaging expansile pons
NSGCT: bHCG/AFP
Optic pathway glioma in NF1

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8
Q

Pilocytic astrocyoma mutations

A

BRAF-KIAA1549 fusion (60-80%
BRAF V600E (10-15%)

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9
Q

CNS GCT tumor markers

A

Choriocarcinoma: bHCG
NGGCT: bHCG, AFP
embryonal carc or yolk sac: AFP
syncytiotrophoblastic germinoma: bHCG

pure germinoma or mature teratoma: negative all

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10
Q

CNS germinoma 2nd look indications

A

residual disease (>1.5cm)
progressive disease w normal tumor markers

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11
Q

CNS germinoma vs NGGCT tx, px

A

Germinoma:
- biopsy
- chemo x4 (carbo/etop)
- +- 2nd look
- response based RT, minimum whole ventricular

NGGCT:
- optional biopsy (nonsecreting)
- chemo x6
- CSI if no viable tumor
- HDC + ASCT if viable tumor

Germinoma 5yDFS >90%
NGGCT: EFS 80-90%

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12
Q

ependymoma px factors

A

Poor:
- <GTR
- no RT
- younger age
- PF-A 1q gain
- 6q loss
- anaplastic histo
- PF-A and RELA

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13
Q

ependymoma grading

A

grade I: subependymoma; rare in children
grade I: myxopapillary
grade II: papillary, clear cell, tancytic
grade II-III: RELA fusion, anaplastic

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14
Q

CNS elevated bHCG DDx

A

syncytiotrophoblastic germinoma
choriocarcinoma
mixed GCT
NGGCT (with AFP)
immature teratoma

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15
Q

CNS elevated AFP DDx

A

NGGCT (w bHCG)
embryonal carcinoma
yolk sac tumor
mixed GCT
immature teratoma

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16
Q

midline posterior fossa mass DDx

A

medulloblastoma (more likely group 3 or 4)
DIPG
glioma
CNS GCT

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17
Q

NF1 optic pathway glioma mutation and tx

A

BRAF V600E

vinblastine
carbo/VCR
MEK inhibitor (trametinib)
BRAF inhibitor (vemurafenib)

if not NF1:
TPCV: thio, procarbazine, CCNU, VCR

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18
Q

Ependymoma mutations and tx

A

PF-A: 1q gain, 6q loss
Supratentorial: RELA fusion, YAP1 fusion

GTR –> RT (focal 59.4 Gy)

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19
Q

Medulloblastoma px by subgroup

A

1) WNT: >90%
2) SHH: 80% if desmoplastic/nodula, <50% if LC/A
3) Group 4: 75%
4) Group 3: 50-70%

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20
Q

Medulloblastoma tx (incl RT doses)

A

GTR

adj RT
- CSI 23.4 Gy (if mets 36 Gy)
- involved field boost to 54 Gy

adj chemo: VCR, carbo, cyclo, etop, cisplat, MTX

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21
Q

DIPG imaging findings

A

midline
>50% of pons
hyperdense
MR hyperintense (dark T1, bright T2)

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22
Q

DIPG mutation if biopsied

A

H3K27M mutation (80-90%
H3K27me3

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23
Q

DIPG tx and px

A

palliative RT: focal 54Gy
symptomatic CS

median survival 10 mo

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24
Q

posterior fossa syndrome features

A

onset 1-3d post op, peak 2w

mutism (transient)
emotional lability
hypotonia
oropharyngeal dysfxn
dysphagia
CN palsies

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25
Q

RT S/E acute, subacute, late

A

acute: fatigue, N/V, skin/hair, h/a

subacute: somnolence, pseudoprogression, edema

late
- brain necrosis
- SMN: glioma, meningioma, 4-5% at 10y
cognitive
- ototox
- cerebrovascular disease
- endo: GH defic, delayed puberty
- infertility

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26
Q

HGG mutations

A

H3K27 mutation
adolescents: IDH1 mutation

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27
Q

CNS tumor epi

A

Astrocytoma 40-50%
medullo 20%
ependymoma 10%
HGG 10%
craniopharyngioma 5-10%
choroid plexus 1-2%
ATRT 1-2%
GCT 1-3%

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28
Q

cerebral hemisphere tumor DDx

A

glioma
meningioma
ETMR (previously PNET)
anaplastic astrocytoma

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29
Q

pineal tumor DDx

A

GCT (until proven otherwise)
pineoblastoma
astrocytoma

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30
Q

sellar/suprasellar tumor DDx

A

craniopharyngioma
GCT
optic glioma

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31
Q

cerebellar tumor DDx

A

medullo
ependymoma
cerebellar astrocytoma (JPA)

32
Q

brain stem tumor DDx

A

medullo
JPA
ETMR (previously PNET)

33
Q

infant posterior fossa tumor DDx

A

medullo
DIPG
LGG
Ependymoma
ATRT

34
Q

ventricular tumor DDx

A

CPP
CPC
neurocytoma

35
Q

spinal cord tumor DDx

A

astrocytoma
ependymoma
extradural: NBl, ewing, neurofibroma

36
Q

NF2 CNS tumors

A

acoustic schwannoma (bilat)
meningioma
ependymoma

37
Q

LFS CNS tumors

A

medullo
GBM
HGG

38
Q

FAP (Turcot) CNS tumors

A

medullo (WNT)
GBM
HGG
PNET

39
Q

Gorlin CNS tumors

A

desmoplastic medullo
meningioma
BCC

40
Q

T21 and Klinefelter CNS tumors

A

germinoma

41
Q

DICER-1 CNS tumor

A

pineoblastoma

42
Q

ATRT px factors

A

poor
<3yo
infratentorial
disseminated
germline mutation (SMARCB1)

43
Q

myxopapillary ependymoma features and tx

A

WHO Grade 1
classically at conus

GTR + RT

44
Q

define parinaud syndrome, and DDx

A

bilateral upgaze palsy
convergence nystagmus
light near dissociation
lid retraction (Collier sign)

GCT (germinoma)
choriocarcinoma
immature teratoma

45
Q

LGG path and DDx

A

WHO I-II
lack of aggressive features

pilocytic astrocytoma
SEGA
ganglioglioma
oligodendroglioma
DNET (dysembryoplastic neuroepithelial tumor)
DIG (desmoplastic infantile ganglioglioma)

46
Q

LGG locations

A

cerebellum (15-25%)
cerebral (10-15%)
deep midline (10-15%)
optic pathway (5%)
brainstem (2-4%)

47
Q

LGG staging

A

MR head/spine
Biopsy - not if NF-1
(no CSF)

no metastatic potential

48
Q

LGG genetics and targeted tx

A

BRAF-KIAA1549 fusion
BRAF V600E

MEK or BRAF inhibitor

49
Q

LGG tx, px

A

GTR

STR –>
weekly vinblastine
carbo/VCR
MEK/BRAF inhibitor

NF1: observation
SEGA: mTOR

GTR 5yPFS >90%

50
Q

HGG tx

A

resection
focal RT
+- chemo - ?VCR/carbo

51
Q

Ependymoma tx

A

GTR
RT - focal or CSI if CSF+

52
Q

NF1 OPG epi

A

NF-1: 15-20% have LGG
OPG: 70% have NF-1

53
Q

Diencephalic syndrome

A

diencephalon location
typically young children

happy FTT
hyperkinesis
macrocephaly
neuro: visual field defect, endocrine dysfxn, behaviour change, h/a

54
Q

2yo with 4 mo of FTT NYD. Recent vomiting and nystagmus. MRI reveals large hypothalamic mass with associated hydrocephalus. Syndrome?

A

Diencephalic syndrome

55
Q

List high grade gliomas

A

anaplastic astrocytoma
anaplastic oligodendroglioma
GBM
mixed glial tumors
DIPG
pleomorphic xanthoastrocytoma

56
Q

LGG vs HGG locations

A

LGG: 20% cerebellar; hemispheric, optic pathway, brainstem

HGG: hemispheric, midline

57
Q

HGG predispositions

A

GBM: NF-1, LFS, Turcot
Anaplastic astrocytoma: NF-1, LFS, Turcot, TS

Hx RT

58
Q

GBM imaging

A

thick irregular margin
necrotic core
+- hemorrhage

59
Q

Craniopharyngioma imaging

A

sellar/suprasellar
rim calcifications
cystic
enhancing

60
Q

ATRT histopathology

A

SRBC
loss of INI-1 (SMARCB1)

Germline SMARCB1 mutation 35% (80% <1yo)

61
Q

EMRT epi, tx, px

A

presentation ~3yo
2/3 supratentorial
1/2 mets

max safe resection
CSI
HD chemo w ASCT

very poor - 5yOS 15-35%

62
Q

ATRT epi, ix

A

median age 1y (2/3 <3yo)
anywhere in brain
30% mets (including kidney, skin, liver)

MR brain and spine
CSF
AUS

63
Q

ATRT tx, px

A

GTR
+-2nd look
chemo
+-HD chemo w ASCT
RT (CSI if disseminated)

4yEFS 37%, OS 43%

64
Q

pineoblastoma location, presentation, genetics, px

A

supratentorial
hydrocephalus, Parinaud
RB1, DICER1 germline
OS 50-60%

65
Q

choroid plexus tumor location

A

75% lateral ventricle
15% 4th ventricle
10% 3rd ventricle

66
Q

CPP vs CPC grade, tx

A

CPP: grade I
aCPP: grade II
CPC: grade III-IV (20-40%)

CPP: Sx (GTR alone)
aCPP: Sx -> chemo

CPC:
- Sx
- +-2nd look
- chemo (ICE)
- +- RT if <CR

67
Q

GCT location + presentation, mets?

A

50% pineal (Parinaud syndrome)
40% suprasellar (DI, altered puberty, visual defect)

mets 30%

68
Q

Ependymoma location, mets

A

anywhere
70% infratentorial
30% supratentorial
rare spinal

10-15% mets

69
Q

Ependymoma px factors

A

poor:
subtotal resection
PF-A +1q
-6q
younger
anaplastic
less/no RT

70
Q

Ependymoma tx, px

A

GTR
focal RT (CSI if disseminated)

EFS 60-80%
- PF-A worse (PFS 33% OS 70%)
- PF-B better (PFS 63% OS 100%)
- YAP1 better (PFS 66%, OS 100%)
- RELA worse (PFS 30% OS 75%

71
Q

DIPG presentation

A

triad
- long tract signs
- contralateral CN palsy
- ataxia

72
Q

neuro GCT px

A

Good risk >90%
- germinoma, mature teratoma

int px 70%

poor risk 40%
- choriocarcinoma, yolk sac, embryonal

73
Q

which CNS tumors need CSF for staging

A

MEGA:
- Medullo
- Ependymoma
- GCT
- ATRT

74
Q

summarize ependymoma

A

10% peds CNS
infant PF, old supratentorial
mets 10-15%

PF-A, PF-B, RELA, YAP1

GTR -> focal RT (CSI if CNS+)

px 60-80%
Poor px: PF-A, YAP1, +1q, -6p

75
Q

choroid plexus imaging

A

nonobstructive hydrocephalus
cauliflower appearance
++ enhancement

76
Q

CPC genetics

A

25-45% TP53

H/O RC (31 decks)

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