CHILDHOOD CANCER REPOSITORY

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Patient Derived Xenografts (PDXs)

RequestPDX Phase of
Therapy		Sample
Type		 StageMYCN*ALKTERT
mRNA		TMMTp53WESRNA
Seq		Cell Line (CL)Cyclo +
Topo		Injection
Type
COG-N-415x PD-PMBlood4397F1174L+TERTWT✓✓COG-N-415✓SQ
COG-N-421x PD-PMBlood4220WT+TERTWT✓✓COG-N-421✓SQ
COG-N-424x DxTumor 4198WT+TERTWT✓✓-✓SQ
COG-N-440x PD-PMBlood4300WT+TERTWT✓✓COG-N-440✓SQ & TV
COG-N-452x«PD-PMBlood4156F1174L+TERTWT ✓COG-N-452✓SQ
COG-N-453x«PD-PMTumor4 WT+TERTWT ✓COG-N-453 SQ
COG-N-470x PD-PMBlood42F1174L+TERT  ✓COG-N-470✓SQ
COG-N-471x PD-PMBlood4228WT+TERTWT✓✓COG-N-471✓SQ
COG-N-480x DxBM4159WT-ESTWT✓✓COG-N-480h2✓SQ & TV
COG-N-496x DxBM4104WT+TERTWT✓✓COG-N-496✓SQ
COG-N-518x DxBM45  EST  ✓COG-N-518h SQ
COG-N-519x PD-PMBlood4775WT+TERTWT✓✓COG-N-519✓SQ
COG-N-546x DxBM4334     ✓COG-N-546 SQ
COG-N-549x PDTumor42F1174L+TERTWT✓✓COG-N-549✓SQ
COG-N-557x DxTumor4 F1245L+TERTWT✓✓COG-N-557✓SQ
COG-N-560x DxTumor 4 WT+TERTWT✓✓-✓SQ
COG-N-561x PD-PMBlood4 F1245I+TERTWT✓✓COG-N-561✓SQ & TV
COG-N-564x PD-PMBlood4 WT+TERTWT✓✓COG-N-564h SQ
COG-N-573x DxBM4  +TERT  ✓COG-N-573✓SQ & TV
COG-N-579x PDBM4      ✓COG-N-579✓SQ
COG-N-589x DxTumor4 WT - ALTWT✓✓COG-N-589 SQ
COG-N-590x DxTumor4 WT+TERTWT✓✓COG-N-590✓SQ
COG-N-603x«DxTumor4 WT+TERTWT✓✓COG-N-603✓SQ
COG-N-615x PD-PMBlood4  +TERT  ✓COG-N-615 SQ
COG-N-618x«PDBM4 WT+TERTWT✓✓COG-N-618✓SQ
COG-N-619x«PDBM4 WT+TERTWT✓✓COG-N-619 SQ
COG-N-620x PDTumor4 WT-ALTWT✓✓- SQ
COG-N-623x«PDTumor4 WT+TERTWT✓✓COG-N-623 SQ
COG-N-624x PDTumor4 WT+TERTWT✓✓- SQ
COG-N-625x PDTumor4 WT - ALTWT✓✓COG-N-625hnb✓SQ
COG-N-627x DxTumor4      ✓COG-N-627hnb SQ
COG-N-628x DxBM4 WT+TERTWT✓✓COG-N-628✓SQ
COG-N-636x DxTumor4  +TERT  ✓COG-N-636✓SQ
COG-N-649x DxTumor4  -EST ✓✓- SQ
COG-N-653x DxTumor4      ✓COG-N-652h✓SQ
COG-N-660x PD-PMBlood4      ✓COG-N-660hnb SQ
COG-N-669x PD-PMBlood4   ALT  ✓- SQ
COG-N-672x PD-PMBlood4      ✓COG-N-672✓SQ
COG-N-676x PDTumor4      ✓- SQ
COG-N-677x PDBM4      ✓- SQ
COG-N-682x DxBM4      ✓COG-N-682 SQ
COG-N-683x DXBM4      ✓COG-N-683✓SQ
COG-N-685x PD-PMBlood4      ✓COG-N-685 SQ
COG-N-695x DxTumor4      ✓- SQ
COG-N-697x DxTumor4      ✓COG-N-697h SQ
COG-N-699x DxTumor4   ALT  ✓- SQ
COG-N-700x DxTumor4   ALT  ✓COG-N-700 SQ
Felix-PDX PD-PMBlood43F1245C+ R342✓✓Felix S


1 - Phase of therapy for the patient at the time the specimen used to derive the cell line was obtained
Dx (Diagnosis): cell lines derived from samples of patients prior to treatment
PD (Progressive Disease): cell lines derived from samples of patients who relapsed after chemotherapy
PD-BMT: cell lines derived from samples of patients who relapsed after chemotherapy and bone marrow transplantation
PD-PM: cell lines derived from samples of post-mortem patients who relapsed after chemotherapy

2 -MYCN oncogene amplification
N: MYCN Non-Amplified
A: MYCN Amplified

3 - TP53 gene function
F: Functional
N: Non-Functional

4 - EWS/FLI1 Status
FLI1: friend leukemia insertion - chromosome 11, t(11;22)
ERG: ets-related gene - chromosome 21, t(21;22)

ALK mutation

WT: Wild Type

TMM:Telomere Maintenance mechanism

✓ = Available/available upon request

SQ- subcutaneous injection

TV- intravenous (tail vein)

EST- ever shorter telomeres, i.e. telomerase and ALT negative

TERT+- telomerase positive

ALT - alternate lengthening of telomeres, C-circle positive

Cyclo + Topo - PDX was assessed for response in mice to cyclophosphamide + topotecan.