COG 2019-2022 Flashcards

1
Q

NBL 4S
- how much MYCA
- px

A

11% 4S are MYCA
- more likely unfav histo
worse px (44 vs 68%)

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2
Q

R/R NBl tx, px, tox

A

Irinotecan, temozolamide, dinutuximab+GMCSF
40% PR/CR
1yPFS/OS 68/84%
tox: fever/inf, neutropenia, pain, diarrhea

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3
Q

HR NBl RT boost?

A

21.6 Gy to preop PTV
NO boost to gross residual

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4
Q

HR NBl dinutuximab outcomes

A

5yEFS/OS 61/72%
CR or VGPR better EFS than PR but no OS difference

higher dinutuximab peak = better EFS

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5
Q

heterogeneous vs homogeneous MYCNa

A

same EFS/OS
heterogeneous: more thoracic primary, less 1pLOH

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6
Q

RMS PET utility

A

IR/HR PET response doesn’t correlate with EFS/OS

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7
Q

ARMS group 1 (complete resection) need adj RT?

A

YES RT if fusion positive - RT improves outcomes 78 vs 17%.

fusion negative no diff

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8
Q

RMS group 3 delayed primary excision?

A

DPE reduces RT dose and improves OS

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9
Q

RMS Group 3 do you need CR?

A

No. CR improves EFS but not OS

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10
Q

Paratesticular RMS staging and px

A

10+yo paratesticular needs RPLND
goal 7-12 LN
5yEFS 92%

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11
Q

biliary RMS tx and px

A

classically LR tx (resection only). But suboptimal outcomes - 5yEFS/OS 70/76%

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12
Q

RMS anaplasia impact

A

not independent px factor

may be surrogate for TP53 mutations

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13
Q

synovial sarcoma outcomes

A

5yEFS/OS
LR 90/98%
IR 70/89%
HR 8/13%

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14
Q

Can you decrease IT MTX intensity for nodular desmoplastic or MBEN

A

ACNS1221 - closed early

can’t remove IT MTX - higher relapsed rate

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15
Q

ATRT RT timing and px

A

timing doesn’t impact survival
4yEFS/OS 37/43%

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16
Q

CNS NGGCT RT type

A

proton WVRT (lower doses to normal brain structures)

17
Q

asparaginase discontinuation impact on ALL px

A

HR - lower DFS (HR 1.5)
SR - no impact (except those with SER)

18
Q

B-ALL relapse, late BM or early CNS. VCR dosing, px factor, and HSCT utility

A

VCR intensive dosing closed early due to worse toxicity

EOI1MRD + worse EFS/OS (53/60 vs 85/95)

HSCT improved 3yDFS but not OS

19
Q

B-ALL EOC MRD px impact

A

HR: worse DFS (5y 40%)
SR: worse DFS (73 vs 90) but same OS (90 vs 95)

20
Q

AML dexrazoxane impact

A

Improves EF/SF (HR 0.55) with no change in outcome

21
Q

AML1031 bortezomib impact

A

no improvement and increased tox (peripheral neuropathy and ICU admissions)
3yEFS/OS 45/65%

22
Q

acute erythroid leukemia - mutation and px

A

NUP98 fusions enriched (32%)
5yEFS 20 vs 46, OS 20 vs 66

23
Q

R/R AML immunophenotype and mutations

A

CD123
enriched KMT2A and FLT3
inferior outcomes

24
Q

AAML1031 FLT3 result incl tox

A

sorafenib independent benefit (induction and sgl agent maintenance)
tox: rash, hand-foot syndrome, fever

25
Q

AAML1031 induction II intensification?

A

mitox/cytarabine had increased tox and no benefit over ADE

26
Q

LR AML tx

A

LR-1 (fav cyto and MRD-) 4 courses instead of 5

27
Q

Ewing Sarcoma newly identified IHC

A

STAG2 loss of expression - worse px (5yEFS 54 vs 75%)

28
Q

Osteo TP53 mutations

A

5% germline mutation
- 1/2 de novo

29
Q

HL SMN rate, types, RF

A

10y cumulative incidence 1.3%
AML, solid, NHL
RF RT, B symptoms, race

30
Q

HL HR type of RT

A

response adapted rather than IFRT

31
Q

AHEP0731 SCU impact on outcome

A

typically makes Stage I-II IR
BUT SCU no impact on outcome

32
Q

HB type of biopsy

A

percutaneous is best - same yield and no hemorrhage

33
Q

HB pure fetal tx/px

A

resection only
5yEFS/OS 100%

34
Q

WT epithelial predominant histo stage 1 tx/px

A

fav histo
4yEFS/OS 96/100% regardless of observation or EE4A

35
Q

features of WT vs nephrogenic rests

A

WT: spherical, exophytic, >1.75cm
rests: homogeneity = perilobar, inhomogeneous = intralobar

36
Q

Diffuse hyperplastic perilobar nephroblastomatosis tx, px (incl risk of WT progression)

A

EE4A
5yOS 100%
25% progress to WT

37
Q

RCC tx, px

A

complete resection only even in LN+
4yEFS/OS 80/84%

38
Q

Dexrazoxane diseases and outcome

A

ALL HL osteo
effective
not associated with relapse, SMN, mortality