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Conference Paper: Reclassification of central nervous system primitive neuroectodermal tumor (CNS-PNET) into entities reflects outcome: results from the prospective SJYC07 and SJMB03 trials

TitleReclassification of central nervous system primitive neuroectodermal tumor (CNS-PNET) into entities reflects outcome: results from the prospective SJYC07 and SJMB03 trials
Authors
Keywordschemotherapy regimen
heterogeneity
choroid plexus neoplasms
dna methylation
ganglioneuroblastoma
Issue Date2018
PublisherOxford University Press. The Journal's web site is located at http://neuro-oncology.dukejournals.org
Citation
The 18th International Symposium on Pediatric Neuro-Oncology (ISPNO 2018), Denver, Colorado, USA, 30 June – 3 July 2018. Abstracts in Neuro-Oncology, 2018, v. 20 n. Suppl. 2, p. i71-i72, article no. EMBR-14 How to Cite?
AbstractBACKGROUND: Central nervous system primitive neuroectodermal tumor (CNS-PNET) was removed from the WHO classification after DNA-methylation profiling unveiled its underlying heterogeneity. Here we describe the makeup and outcome of patients histopathologically diagnosed as CNS-PNET treated on 2 multi-center, prospective trials. METHODS: Patients <3yr received chemotherapy with/without focal irradiation (SJYC07). Patients ≥3yr received risk-adapted craniospinal-irradiation (23.4Gy for localized disease, 36–39.6Gy for metastatic) and chemotherapy (SJMB03). DNA-methylation was performed using Infinium MethylationEPIC BeadChip and profiled on DKFZ molecularneuropathology2.0 classifier. RESULTS: Fifty-six patients were enrolled (SJYC07=32; SJMB03=24) with median age 2.86yr (range: 0.64–19.47). Sixteen were stratified as high-risk including 10 with metastasis. Histopathological diagnosis upon central review was CNS-PNET (n=34), ETMR (n=17), CNS-neuroblastoma/ganglioneuroblastoma (n=3) and HGNET (n=2). 42/56 cases were methylation-profiled into ETMR (N=13), GBM (N=3), MBgroup3 (N=2), CNS-NB-FOXR2 (N=3), CNS-EFT-CIC (N=1), EPN-RELA (N=1), choroid plexus tumor (pediatric B) (N=1), PBgroupB (N=1), normal tissue (N=3), and “no match” (calibrated scores <0.9) (N=14). Median duration of follow-up was 2.24yr (range: 0.06–12.7). 5yr-OS/PFS in SJMB03-AR and -HR patients were 46.7 ± 12.9%/46.7 ± 12.9% and 33.3 ± 15.7%/33.3 ± 15.7% (OS p=0.503; PFS p=0.494). 5yr-OS/PFS in SJYC07-IR and -HR patients were 46.8 ± 10.6%/44.7 ± 10.4% and 57.1 ± 18.7%/14.3 ± 13.2% respectively (OS p=0.973; PFS p=0.046). Patients methylation-profiled as ETMR/GBM/MB had 5yr-OS/PFS of 24.1 ± 10.4%/14.8 ± 8.9% compared to 90.0 ± 9.5%/80.0 ± 12.6% in the other entities (OS p=0.001; PFS p<0.001). Age, metastasis, extent of surgery and treatment protocol did not significantly impact outcome. Classification by DNA-methylation profiling (p=0.037), histopathological diagnosis (p=0.019) and radiation use (p<0.001) were predictive of PFS. CONCLUSION: Outcome of pediatric CNS-PNET varied but better conformed to convention when assigned a new entity.
Descriptionposter presentation
Persistent Identifierhttp://hdl.handle.net/10722/265184
ISSN
2019 Impact Factor: 10.247
2015 SCImago Journal Rankings: 3.196

 

DC FieldValueLanguage
dc.contributor.authorLiu, APY-
dc.contributor.authorOrr, B-
dc.contributor.authorLin, T-
dc.contributor.authorHassall, T-
dc.contributor.authorBowers, DC-
dc.contributor.authorBouffet, E-
dc.contributor.authorGururangan, S-
dc.contributor.authorFisher, P-
dc.contributor.authorCrawford, J-
dc.contributor.authorKellie, SJ-
dc.contributor.authorChintagumpala, M-
dc.contributor.authorFisher, M-
dc.contributor.authorBendel, A-
dc.contributor.authorEllison, D-
dc.contributor.authorRobinson, G-
dc.contributor.authorGajjar, A-
dc.date.accessioned2018-11-20T02:01:46Z-
dc.date.available2018-11-20T02:01:46Z-
dc.date.issued2018-
dc.identifier.citationThe 18th International Symposium on Pediatric Neuro-Oncology (ISPNO 2018), Denver, Colorado, USA, 30 June – 3 July 2018. Abstracts in Neuro-Oncology, 2018, v. 20 n. Suppl. 2, p. i71-i72, article no. EMBR-14-
dc.identifier.issn1522-8517-
dc.identifier.urihttp://hdl.handle.net/10722/265184-
dc.descriptionposter presentation-
dc.description.abstractBACKGROUND: Central nervous system primitive neuroectodermal tumor (CNS-PNET) was removed from the WHO classification after DNA-methylation profiling unveiled its underlying heterogeneity. Here we describe the makeup and outcome of patients histopathologically diagnosed as CNS-PNET treated on 2 multi-center, prospective trials. METHODS: Patients <3yr received chemotherapy with/without focal irradiation (SJYC07). Patients ≥3yr received risk-adapted craniospinal-irradiation (23.4Gy for localized disease, 36–39.6Gy for metastatic) and chemotherapy (SJMB03). DNA-methylation was performed using Infinium MethylationEPIC BeadChip and profiled on DKFZ molecularneuropathology2.0 classifier. RESULTS: Fifty-six patients were enrolled (SJYC07=32; SJMB03=24) with median age 2.86yr (range: 0.64–19.47). Sixteen were stratified as high-risk including 10 with metastasis. Histopathological diagnosis upon central review was CNS-PNET (n=34), ETMR (n=17), CNS-neuroblastoma/ganglioneuroblastoma (n=3) and HGNET (n=2). 42/56 cases were methylation-profiled into ETMR (N=13), GBM (N=3), MBgroup3 (N=2), CNS-NB-FOXR2 (N=3), CNS-EFT-CIC (N=1), EPN-RELA (N=1), choroid plexus tumor (pediatric B) (N=1), PBgroupB (N=1), normal tissue (N=3), and “no match” (calibrated scores <0.9) (N=14). Median duration of follow-up was 2.24yr (range: 0.06–12.7). 5yr-OS/PFS in SJMB03-AR and -HR patients were 46.7 ± 12.9%/46.7 ± 12.9% and 33.3 ± 15.7%/33.3 ± 15.7% (OS p=0.503; PFS p=0.494). 5yr-OS/PFS in SJYC07-IR and -HR patients were 46.8 ± 10.6%/44.7 ± 10.4% and 57.1 ± 18.7%/14.3 ± 13.2% respectively (OS p=0.973; PFS p=0.046). Patients methylation-profiled as ETMR/GBM/MB had 5yr-OS/PFS of 24.1 ± 10.4%/14.8 ± 8.9% compared to 90.0 ± 9.5%/80.0 ± 12.6% in the other entities (OS p=0.001; PFS p<0.001). Age, metastasis, extent of surgery and treatment protocol did not significantly impact outcome. Classification by DNA-methylation profiling (p=0.037), histopathological diagnosis (p=0.019) and radiation use (p<0.001) were predictive of PFS. CONCLUSION: Outcome of pediatric CNS-PNET varied but better conformed to convention when assigned a new entity.-
dc.languageeng-
dc.publisherOxford University Press. The Journal's web site is located at http://neuro-oncology.dukejournals.org-
dc.relation.ispartofNeuro-Oncology-
dc.relation.ispartofThe 18th International Symposium on Pediatric Neuro-Oncology (ISPNO 2018)-
dc.subjectchemotherapy regimen-
dc.subjectheterogeneity-
dc.subjectchoroid plexus neoplasms-
dc.subjectdna methylation-
dc.subjectganglioneuroblastoma-
dc.titleReclassification of central nervous system primitive neuroectodermal tumor (CNS-PNET) into entities reflects outcome: results from the prospective SJYC07 and SJMB03 trials-
dc.typeConference_Paper-
dc.identifier.emailLiu, APY: apyliu@hku.hk-
dc.identifier.authorityLiu, APY=rp01357-
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1093/neuonc/noy059.198-
dc.identifier.hkuros295913-
dc.identifier.hkuros315013-
dc.identifier.volume20-
dc.identifier.issueSuppl. 2-
dc.identifier.spagei71-
dc.identifier.epagei72-
dc.publisher.placeUnited States-

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