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%