COG 2019-2022

Question 1

NBL 4S
- how much MYCA
- px

Answer 1

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

Question 2

R/R NBl tx, px, tox

Answer 2

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

Question 3

HR NBl RT boost?

Answer 3

21.6 Gy to preop PTV
NO boost to gross residual

Question 4

HR NBl dinutuximab outcomes

Answer 4

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

higher dinutuximab peak = better EFS

Question 5

heterogeneous vs homogeneous MYCNa

Answer 5

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

Question 6

RMS PET utility

Answer 6

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

Question 7

ARMS group 1 (complete resection) need adj RT?

Answer 7

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

fusion negative no diff

Question 8

RMS group 3 delayed primary excision?

Answer 8

DPE reduces RT dose and improves OS

Question 9

RMS Group 3 do you need CR?

Answer 9

No. CR improves EFS but not OS

Question 10

Paratesticular RMS staging and px

Answer 10

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

Question 11

biliary RMS tx and px

Answer 11

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

Question 12

RMS anaplasia impact

Answer 12

not independent px factor

may be surrogate for TP53 mutations

Question 13

synovial sarcoma outcomes

Answer 13

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

Question 14

Can you decrease IT MTX intensity for nodular desmoplastic or MBEN

Answer 14

ACNS1221 - closed early

can’t remove IT MTX - higher relapsed rate

Question 15

ATRT RT timing and px

Answer 15

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

Question 16

CNS NGGCT RT type

Answer 16

proton WVRT (lower doses to normal brain structures)

Question 17

asparaginase discontinuation impact on ALL px

Answer 17

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

Question 18

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

Answer 18

VCR intensive dosing closed early due to worse toxicity

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

HSCT improved 3yDFS but not OS

Question 19

B-ALL EOC MRD px impact

Answer 19

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

Question 20

AML dexrazoxane impact

Answer 20

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

Question 21

AML1031 bortezomib impact

Answer 21

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

Question 22

acute erythroid leukemia - mutation and px

Answer 22

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

Question 23

R/R AML immunophenotype and mutations

Answer 23

CD123
enriched KMT2A and FLT3
inferior outcomes

Question 24

AAML1031 FLT3 result incl tox

Answer 24

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

Question 25

AAML1031 induction II intensification?

Answer 25

mitox/cytarabine had increased tox and no benefit over ADE

Question 26

LR AML tx

Answer 26

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

Question 27

Ewing Sarcoma newly identified IHC

Answer 27

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

Question 28

Osteo TP53 mutations

Answer 28

5% germline mutation - 1/2 de novo

Question 29

HL SMN rate, types, RF

Answer 29

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

Question 30

HL HR type of RT

Answer 30

response adapted rather than IFRT

Question 31

AHEP0731 SCU impact on outcome

Answer 31

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

Question 32

HB type of biopsy

Answer 32

percutaneous is best - same yield and no hemorrhage

Question 33

HB pure fetal tx/px

Answer 33

resection only 5yEFS/OS 100%

Question 34

WT epithelial predominant histo stage 1 tx/px

Answer 34

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

Question 35

features of WT vs nephrogenic rests

Answer 35

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

Question 36

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

Answer 36

EE4A 5yOS 100% 25% progress to WT

Question 37

RCC tx, px

Answer 37

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

Question 38

Dexrazoxane diseases and outcome

Answer 38

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