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Pedes > Pediatric LGG > Flashcards

Pediatric LGG Flashcards

1
Q

Tuberous sclerosis genetics

A

VHL, Gorlin

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2
Q

What do NF-1 patients get

A

optic pathway gliomas, intracranial glioma, JPA

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3
Q

Describe the characteristics of NF-1 mediated tumors

A

Tumors tend to be low grade, slow-growing.
3x risk of vasculopathy and occlusion of the Circle of Willis (moyamoya syndrome)

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4
Q

What about RT in Nf-1 patients

A

3x increased risk of radiation-induced secondary
malignancy

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5
Q

What tumors do NF-2 patients get

A

bilateral acoustic neuromas, spinal ependymoma

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6
Q

What should primary tx be in patients with tumors and neurofibromatosis

A

Treat with vincristine/carboplatin and avoid radiation if possible

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7
Q

What is the workup in pediatric LGG

A

MRI, then max safe resection followed by
MRI 24-72hrs post-surgery

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8
Q

Describe the histolpathology

A

IDH-mutants, 1p/19q-codeleted = oligodendroglioma
1p19q intact = astrocytoma

Grading (WHO)
Grade I = noninfiltrative = pilocytic astrocytomas, pleomorphic xanthoastrocytoma,
subependymal giant cell astrocytomas, ganglioglioma

Grade II = infiltrative/diffuse = 1p/19q co-deleted oligodendroglioma; ATRX retained

Grade III = ATRX lost (astrocytoma)

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9
Q

What is the treatment paradigm

A

If initially nonresectable or to delay radiation therapy, then

First line = vincristine/carboplatin – delays radiation by 3 years on average

Second line = vinblastine, TPCV, vinorelbine, irinotecan/avastin are common agents

Surgery- max safe resection

Then RT

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10
Q

What are the RT doses and cvolumes

A

Radiation
o GTV = gross disease (T1+Gad and FLAIR) and cavity
o CTV5040-5400 = GTV + 5mm anatomically constrained
PTV= CTV+ 3mm

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11
Q

What factor must be considered in follow up?

A

Pseudoprogression is quite common in LGG. NOn enhancing tumors may become enhancing after RT and can mimic tumor progression

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12
Q

What are 5 year survival outcomes

A

5 yr OS = 80%
5 yr PFS= 65%

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13
Q

Describe the histological appearance of pilocytic astrocytomas

A

-Well circumscribed, cystic, intensely enhancing solid mural nodules
-Degenerative hyalinization of blood vessels cause their enhancement
-Demonstrate rosenthal fibers

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14
Q

What genetic pathway is commonly altered in piloctyic astrocytoma

A

RAS-RAF_MEK-ERK

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15
Q

What is the treatment paradigm for pilocytic astrocytoma

A

Max safe resection
Consider observation following resection even if microscopically positive margins
- chemotherapy if progressive disease
- also consider observation especially if NF-1 given that they may spontaneously regress
- If unresectable or they fail chemo, then RT

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16
Q

What type of chemotherapy is used for pilocytic astrocytoma

A

vincristine/ carboplatin

17
Q

How long does the use of chemo delay RT by in pilocytic astrocytoma

A

By 3 years

18
Q

Describe RT prescription

A

GTV- T1 post gad enhancing area, surgical cavity, any gross disease and T2 FLAIR
CTV- GTV+ 5 mm, respecting anatomical barriers
PTV- CTV+ 3 mm

19
Q

What genetic pathway is commonly altered in pleomorphic xanthroastrocytoma

A

BRAF V600E mutations and CDKN2A

20
Q

What genetic disease is associated with Subependymal giant cell astrocytomas

A

Tuberous Sclerosis

21
Q

What is the common treatment paradigm of Subependymal giant cell astrocytomas

A

Observation if asymptomatic
If symptomatic, max safe resection and MTOR inhibitors
Avodi RT given high risk of secondary malignancy

22
Q

What is the most common type of optic pathway glioma

A

pilocytic astrocytoma

23
Q

When do optic pathway gliomas present

A

90% present before age of 20
75% present before age of 10

24
Q

What is the

What is the most common symptom of optic pathway glioma

A

painless proptosis

25
Q

What is the diffferential diagnosis of optic pathway glioma

A

retinoblastoma
optic neuritis
lymphoma
harmatoma
meningioma

26
Q

What symptom may indicate faster progression in optic pathway glioma

A

loss of color vision

27
Q

what is the workup for optic pathway glioma

A

fundoscopic exam
visual field testing
cranial nerve exam
optic coherence tomography

28
Q

What is the treatmnet paradaigm in a patient under 7 yrs of age or with NF-1

A

chemo with vincristine/carboplatin to delay RT

29
Q

What is the tx paradigm for a optic pathway glioma pt with BRAF V600 mutation

A

Darafenib/tramtinib

30
Q

What is the blindness free survival with RT first vs. chemo first

A

100% with RT vs. 60% with chemo

31
Q

In which patients would optic pathway glioma regress spontaneously

A

Patients with NF-1

32
Q

Describe the commonly observed long term toxicities after tx of LGG in pedes

A

skull hypoplasia
pituitary failure- GH goes first, then LF/FSH, then TSH
IQ defecits
vasculopathy- moya moya
secondary malignancy- 10% but can be 15-20% in NF-1

Pedes (6 decks)

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