COG 2019-2022 Flashcards
NBL 4S
- how much MYCA
- px
11% 4S are MYCA
- more likely unfav histo
worse px (44 vs 68%)
R/R NBl tx, px, tox
Irinotecan, temozolamide, dinutuximab+GMCSF
40% PR/CR
1yPFS/OS 68/84%
tox: fever/inf, neutropenia, pain, diarrhea
HR NBl RT boost?
21.6 Gy to preop PTV
NO boost to gross residual
HR NBl dinutuximab outcomes
5yEFS/OS 61/72%
CR or VGPR better EFS than PR but no OS difference
higher dinutuximab peak = better EFS
heterogeneous vs homogeneous MYCNa
same EFS/OS
heterogeneous: more thoracic primary, less 1pLOH
RMS PET utility
IR/HR PET response doesn’t correlate with EFS/OS
ARMS group 1 (complete resection) need adj RT?
YES RT if fusion positive - RT improves outcomes 78 vs 17%.
fusion negative no diff
RMS group 3 delayed primary excision?
DPE reduces RT dose and improves OS
RMS Group 3 do you need CR?
No. CR improves EFS but not OS
Paratesticular RMS staging and px
10+yo paratesticular needs RPLND
goal 7-12 LN
5yEFS 92%
biliary RMS tx and px
classically LR tx (resection only). But suboptimal outcomes - 5yEFS/OS 70/76%
RMS anaplasia impact
not independent px factor
may be surrogate for TP53 mutations
synovial sarcoma outcomes
5yEFS/OS
LR 90/98%
IR 70/89%
HR 8/13%
Can you decrease IT MTX intensity for nodular desmoplastic or MBEN
ACNS1221 - closed early
can’t remove IT MTX - higher relapsed rate
ATRT RT timing and px
timing doesn’t impact survival
4yEFS/OS 37/43%
CNS NGGCT RT type
proton WVRT (lower doses to normal brain structures)
asparaginase discontinuation impact on ALL px
HR - lower DFS (HR 1.5)
SR - no impact (except those with SER)
B-ALL relapse, late BM or early CNS. VCR dosing, px factor, and HSCT utility
VCR intensive dosing closed early due to worse toxicity
EOI1MRD + worse EFS/OS (53/60 vs 85/95)
HSCT improved 3yDFS but not OS
B-ALL EOC MRD px impact
HR: worse DFS (5y 40%)
SR: worse DFS (73 vs 90) but same OS (90 vs 95)
AML dexrazoxane impact
Improves EF/SF (HR 0.55) with no change in outcome
AML1031 bortezomib impact
no improvement and increased tox (peripheral neuropathy and ICU admissions)
3yEFS/OS 45/65%
acute erythroid leukemia - mutation and px
NUP98 fusions enriched (32%)
5yEFS 20 vs 46, OS 20 vs 66
R/R AML immunophenotype and mutations
CD123
enriched KMT2A and FLT3
inferior outcomes
AAML1031 FLT3 result incl tox
sorafenib independent benefit (induction and sgl agent maintenance)
tox: rash, hand-foot syndrome, fever
AAML1031 induction II intensification?
mitox/cytarabine had increased tox and no benefit over ADE
LR AML tx
LR-1 (fav cyto and MRD-) 4 courses instead of 5
Ewing Sarcoma newly identified IHC
STAG2 loss of expression - worse px (5yEFS 54 vs 75%)
Osteo TP53 mutations
5% germline mutation
- 1/2 de novo
HL SMN rate, types, RF
10y cumulative incidence 1.3%
AML, solid, NHL
RF RT, B symptoms, race
HL HR type of RT
response adapted rather than IFRT
AHEP0731 SCU impact on outcome
typically makes Stage I-II IR
BUT SCU no impact on outcome
HB type of biopsy
percutaneous is best - same yield and no hemorrhage
HB pure fetal tx/px
resection only
5yEFS/OS 100%
WT epithelial predominant histo stage 1 tx/px
fav histo
4yEFS/OS 96/100% regardless of observation or EE4A
features of WT vs nephrogenic rests
WT: spherical, exophytic, >1.75cm
rests: homogeneity = perilobar, inhomogeneous = intralobar
Diffuse hyperplastic perilobar nephroblastomatosis tx, px (incl risk of WT progression)
EE4A
5yOS 100%
25% progress to WT
RCC tx, px
complete resection only even in LN+
4yEFS/OS 80/84%
Dexrazoxane diseases and outcome
ALL HL osteo
effective
not associated with relapse, SMN, mortality
