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Conference Paper: Favorable outcomes in children with pineoblastoma treated with risk-adapted craniospinal irradiation and chemotherapy: results and molecular analysis from the SJYC07 and SJMB03 trials

TitleFavorable outcomes in children with pineoblastoma treated with risk-adapted craniospinal irradiation and chemotherapy: results and molecular analysis from the SJYC07 and SJMB03 trials
Authors
Keywordschemotherapy regimen
heterogeneity
choroid plexus neoplasms
dna methylation
medulloblastoma
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, abstract no. EMBR-13 How to Cite?
AbstractBACKGROUND: Pineoblastoma (PB) is an aggressive embryonal tumor that has been uniformly treated with high-dose craniospinal irradiation (CSI). Herein, we describe patients diagnosed with PB treated on 2 multi-center, prospective trials with risk-adapted radiation regimens. METHODS: Patients <3yr received chemotherapy with/without focal irradiation (SJYC07). Patients ≥3yr received risk-adapted CSI (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: 40 patients were enrolled (SJMB03=28; SJYC07=12) with 6.07yr median age (range: 0.37–20.4) and 3.8yr median follow-up (range: 0.37–13.1). Twenty-two were stratified as high-risk (HR). All 28 SJMB03 patients received CSI; 6 SJYC07 patients received focal RT and 6 were not irradiated. All but one patient received chemotherapy (parental refusal). On SJMB03, 10/11 non-HR patients receiving 23.4Gy CSI survived without progression (5yr-PFS 100%) as compared to 8/17 HR patients (5yr-PFS 56.3 ± 12.4%). On SJYC07, 11/12 progressed (5yr-PFS 8.3 ± 8.0%). 23/40 tumors (58%) were methylation profiled into the following categories: PBgroupB (N=11), PBgroupA(N=2), pineal parenchymal tumor (N=1), choroid plexus tumor (CPT) (subclass pediatric B) (N=1), with 6 samples having calibrated score <0.9 (associated with medulloblastomagroup3=4, CPT=1, PBgroupB=1, no-match=2). All patients with PB-B were aged ≥3yr with 67% (8/12) surviving progression-free. Patients <3yr had PBgroupA/medulloblastoma/CPT and only 14% (1/7) survived progression-free. CONCLUSION: Non-metastatic PB in children ≥3yr has excellent survival with average-risk medulloblastoma therapy. The poor survival of young children and HR-PB might be explained by underlying molecular heterogeneity, and clustering of pineal tumors with MBgroup3 or CPT warrants further investigation.
Descriptionposter presentation
Persistent Identifierhttp://hdl.handle.net/10722/265182
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.authorBouffet, E-
dc.contributor.authorGururangan, S-
dc.contributor.authorCrawford, J-
dc.contributor.authorKellie, SJ-
dc.contributor.authorChintagumpala, M-
dc.contributor.authorFisher, M-
dc.contributor.authorBowers, DC-
dc.contributor.authorHassall, T-
dc.contributor.authorEllison, D-
dc.contributor.authorRobinson, G-
dc.contributor.authorGajjar, A-
dc.date.accessioned2018-11-20T02:01:43Z-
dc.date.available2018-11-20T02:01:43Z-
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, abstract no. EMBR-13-
dc.identifier.issn1522-8517-
dc.identifier.urihttp://hdl.handle.net/10722/265182-
dc.descriptionposter presentation-
dc.description.abstractBACKGROUND: Pineoblastoma (PB) is an aggressive embryonal tumor that has been uniformly treated with high-dose craniospinal irradiation (CSI). Herein, we describe patients diagnosed with PB treated on 2 multi-center, prospective trials with risk-adapted radiation regimens. METHODS: Patients <3yr received chemotherapy with/without focal irradiation (SJYC07). Patients ≥3yr received risk-adapted CSI (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: 40 patients were enrolled (SJMB03=28; SJYC07=12) with 6.07yr median age (range: 0.37–20.4) and 3.8yr median follow-up (range: 0.37–13.1). Twenty-two were stratified as high-risk (HR). All 28 SJMB03 patients received CSI; 6 SJYC07 patients received focal RT and 6 were not irradiated. All but one patient received chemotherapy (parental refusal). On SJMB03, 10/11 non-HR patients receiving 23.4Gy CSI survived without progression (5yr-PFS 100%) as compared to 8/17 HR patients (5yr-PFS 56.3 ± 12.4%). On SJYC07, 11/12 progressed (5yr-PFS 8.3 ± 8.0%). 23/40 tumors (58%) were methylation profiled into the following categories: PBgroupB (N=11), PBgroupA(N=2), pineal parenchymal tumor (N=1), choroid plexus tumor (CPT) (subclass pediatric B) (N=1), with 6 samples having calibrated score <0.9 (associated with medulloblastomagroup3=4, CPT=1, PBgroupB=1, no-match=2). All patients with PB-B were aged ≥3yr with 67% (8/12) surviving progression-free. Patients <3yr had PBgroupA/medulloblastoma/CPT and only 14% (1/7) survived progression-free. CONCLUSION: Non-metastatic PB in children ≥3yr has excellent survival with average-risk medulloblastoma therapy. The poor survival of young children and HR-PB might be explained by underlying molecular heterogeneity, and clustering of pineal tumors with MBgroup3 or CPT warrants further investigation.-
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.subjectmedulloblastoma-
dc.titleFavorable outcomes in children with pineoblastoma treated with risk-adapted craniospinal irradiation and chemotherapy: results and molecular analysis from the 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/noy059.197-
dc.identifier.hkuros295912-
dc.identifier.hkuros315018-
dc.identifier.volume20-
dc.identifier.issueSuppl. 2-
dc.identifier.spagei71-
dc.identifier.epagei71-
dc.publisher.placeUnited States-

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