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SADL Oncology > Brain tumors > Flashcards

Brain tumors Flashcards

1
Q

Infratentorial tumors

A 
o	Medulloblastoma
o	Ependymoma
o	Cerebellar Astrocytoma (pilocytic)
o	Brainstem gliomas (DIPG)
o	AT/RT
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2
Q

Cerebrum tumors

A 
o	LGG
o	HGG
o	PNET
o	AT/RT
o	Ependymoma
o	CPC/CPP
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3
Q

Sellar tumors

A 
o	Craniopharyngioma
o	LCH (in the stalk)
o	Germ Cell Tumor
o	Adenoma/hamartoma
o	Pilocytic astrocytoma
o	PNET
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4
Q

Pineal tumors

A 
o	Pineoblastoma
o	GCTs
o	Pineal cyst
o	PNET
o	Astrocytomas
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5
Q

Major post-op issues

A

Obstructive hydrocephalus with increased ICP is managed with steroids followed shortly by EVD placement.

Pituitary dysfunction can lead to an Addisonian picture – treat urgently with steroids

SIADH is common postop – treat with fluid restriction. Cerebral salt wasting also occurs (they are hypovolemic)

Seizures can be the result of the primary tumor or of disturbances from surgery.

Cerebellar mutism – risk factors include tumors and surgery of the vermis, younger age, female, larger resection area. Occurs in 25% of “eligible” surgeries and not all recover.

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6
Q

List 6 side effects of brain radiation

A
  • Skin toxicity
  • Alopecia acutely or subacutely
  • Radiation necrosis
  • Cerebral edema
  • Somnolence syndrome
  • Mucositis can occur if radiation volume includes esophagus.
  • Ototoxicity can be a long term side effect
  • Cataracts occur if the lens is included in the radiation field (risk starts at 10 Gy)
  • Retinal toxicity can occur at > 45 Gy
  • Vacular complications can occur at any time
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7
Q

prognostic factors in LGG

A
  • Age (younger patients do worse – potentially because of difficulty in treating)
  • Histology (fibrillary tumors do worse)
  • Extent of resection
    o In a COG study, 8 year PFS/OS for GTR was 90%/99%
    o 8 year PFS/OS for no GTR was 50%/90%
  • Underlying syndrome (patients with NF1 do better)
  • Location (OPGs do better, patients with diencephalic syndrome potentially do worse)
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8
Q

3 situations do you not need a biopsy?

A

Dipg
Gct with elevated tumor markers
Nf-1, particularly optic pathway gliomas

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9
Q

Diencephalic syndrome

A

emaciation, euphoria, emesis

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10
Q

Parinaud Syndrome

A

Sunsetting eyes, enlarged pupils that are poorly responsive to light, poor eye convergence

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11
Q

what are the tumours found in the optic pathway?

A

pilocytic astrocytoma

retinoblastoma

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12
Q

what tumours orginate in the spinal cord?

A

low grade astrocytomas
ependymoma (especially myxopapillary)
AA/GBM

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13
Q

What CNS tumours like to metastasize to the spine?

A

embryonal tumours, germ cell tumours, ependymoma, astrocytoma (less commonly)

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14
Q

NF1 patients are most commonly associated with which tumour?

A

optic pathway gliomas

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15
Q

NF2 patients are predisposed to which tumours?

A

bilateral acoustic neuromas
meningiomas of the brain
spinal cord ependymoma
schwannoma of the dorsal roots of the spinal cord

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16
Q

what CNS tumours are associated in patients with Li-Fraumeni?

A

choroid plexus carcinomas and GBM

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17
Q

tuberous sclerosis patients are associated with which CNS tumours?

A

SEGA’s- subependymal giant cell astrocytomas-respond to everolimus
they also can develop hamartomas

18
Q

patients with Gorlin syndrome are at risk of developing which CNS tumour

A

medulloblastoma

19
Q 
what WHO grades are for the following gliomas?
----Diffuse astrocytoma
—Anaplastic astrocytoma, 
—Glioblastoma,   
--Pilocytic astrocytoma
—Diffuse midline glioma, H3K27M-mutant
A 
-Diffuse astrocytoma, II 
—Anaplastic astrocytoma, III 
—Glioblastoma,  IV 
—Pilocytic astrocytoma I
—Diffuse midline glioma, H3K27M-mutant IV
20
Q

what are unfavourable prognostic features for LGG?

A 
younger age
diffuse histology
inability for complete resection
diencephalic syndrome
intracranial HTN at presentation
metastatic disease
21
Q

what are the treatment options for LGG?

A

in instances of NF1 or asymptomatic can observe
Gross surgical resection can be curative
chemo options are carboplatin/VCR (60weeks) or TPCV-thioguanine, procarbazine, lomustine, VCR (48 weeks)

22
Q

what is standard treatment for HGG?

A

attempt best possible resection
all get 54Gy with temozolamide (as a sensitizer)
can consider chemo-Pred/VCR/CCNU for 48wks

23
Q

name the WHO classification for medulloblastoma

A
  • Medulloblastoma, genetically defined.
  • Medulloblastoma, WNT-activated.
  • Medulloblastoma, sonic hedgehog (SHH)-activated and TP53-mutant.
  • Medulloblastoma, SHH-activated and TP53-wildtype.
  • Medulloblastoma, non-WNT/non-SHH.
  • Medulloblastoma, group 3.
  • Medulloblastoma, group 4.
  • Medulloblastoma, histologically defined.
  • Medulloblastoma, classic.
  • Medulloblastoma, desmoplastic/nodular.
  • Medulloblastoma with extensive nodularity.
  • Medulloblastoma, large cell/anaplastic.
24
Q

what hereditary cancer syndromes are associated with medulloblastoma?

A 
Turcotte Syndrome
Li-Fraumeni
Gorlin
Fanconi
•Rubinstein-Taybi
25
Q

when should CSF be obtained from medulloblastoma patient for staging?

A

CSF done more than 10 days after the original surgery

26
Q

describe the medulloblastoma risk categories

A

Avg Risk-no mets, <1.5cm2 residual tumour, greater than 3yrs old
High risk: positive CSF or MRI, >1.5cm2 of residual tumour, large cell anaplastic histology, <3yrs old

27
Q

describe the 4 molecular subgroups of medulloblastoma

A

WNT: very good prognosis, seen in older children and adults (10-15%)
SHH: those that aren’t TP53 mutated have a good prognosis. You can see the desmoplastic/nodular histologic subtype
Grp 3: children and infants, VERY frequently metastatic Poor outcome, more common in males
Grp 4:: frequently metatstatic, prognosis intermediate, more common in males

28
Q

do patients with medulloblastoma get CSI?

A

yes, all receive CSI as part of standard of care, however those with high risk disease receive 36cGy instead of 23.4cGy

29
Q

how can you differentiate DIPG from astrocytoma of the brainstem?

A

DIPG do not enhance on imaging

lesion without exophytic component are DIPG (those that have exophytic are usually LGG)

30
Q

what genetic subtype of ependymoma is most common in pediatrics

A 
posterior fossa-EPN-PFA 
usually grade 2 or 3
seen in younger children
has a poor prognosis
see a gain of chromosome 1q in 25% cases which is a poor prognostic factor
31
Q

what location is the most common for an ependymoma to present?

A

fourth ventricle, commonly from the floor of the 4th ventricle

32
Q

what supratentorial mutation is most common in ependymoma’s?

A

The largest subset of pediatric supratentorial (ST) ependymomas are characterized by gene fusions involving RELA, a transcriptional factor important in NF-κB pathway activity

33
Q

what are unfavourable prognostic factors in patients with ependymoma?

A 
molecular profile
tumour location
younger age
subtotal resection
anaplastic histology
lower doses of radiation
34
Q

where do germinomas (non-secreting GCT) like to present? Do these tumours require a biopsy?

A

1) suprasellar region-develop DI, panhypopit
2) Pineal region (Parinaud’s, elevated ICP)

these tumours require a biopsy

35
Q

what type of GCT does not require a biopsy?

A

non-germanomatous germ cell tumours are secretory and do not require a biopsy. Either serum or CSF AFB/BHCG are elevated

36
Q

how are NG-GCT treated?

A

given neoadjuvant therapy with carbo/etop alternating with ifos/etop for 6 cycles, then receive response based rads-with CSI

37
Q

Imaging findings of medullo

A

Posterior fossa
Midline in younger, more lateral in older kids
Contrast enhancing and bright on T2Flair

38
Q

prognostic findings for HGG

A

• GTR (hence location)
• Non H3K27M is favourable (3 year survival for mutated tumor <5%)
• IDH1 mutant carries favourable prognosis
• GBM worse than AA (anaplastic astrocytoma, grade 3)
- age (likelihood to radiate)
- MGMT promoter mutations do better

39
Q

What is the characteristic MRI finding of craniopharyngioma?

A
  • occur in the region of the pituitary gland

o “ccc” = cystic + calcifications = craniopharyngioma

40
Q

What is the treatment for craniopharyngiomas?

A
  • excision if possible
  • 50 Gy reduces risk of recurrence - generally xrt only if residual tumor present
  • No established chemo. Intracystic interferon tried.
41
Q

RF for HGG

A

p53
Biallelic mismatch repair deficiency
Prior radiotherapy

42
Q

Treated NG-GCT. Mass growing but markers normal. What is this?

A

Growing teratoma syndrome

SADL Oncology (17 decks)

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