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https://dora.health.qld.gov.au/qldresearchjspui/handle/1/3917
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DC Field | Value | Language |
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dc.contributor.author | Ellison, D. W. | en |
dc.contributor.author | Boop, F. A. | en |
dc.contributor.author | Merchant, T. E. | en |
dc.contributor.author | Gajjar, A. J. | en |
dc.contributor.author | Upadhyaya, S. | en |
dc.contributor.author | Robinson, G. W. | en |
dc.contributor.author | Orr, B. | en |
dc.contributor.author | Onar-Thomas, A. | en |
dc.contributor.author | Billups, C. A. | en |
dc.contributor.author | Armstrong, G. T. | en |
dc.contributor.author | Sadighi, Z. S. | en |
dc.contributor.author | Hassall, T. | en |
dc.contributor.author | Bowers, D. C. | en |
dc.contributor.author | Bendel, A. E. | en |
dc.contributor.author | Crawford, J. R. | en |
dc.contributor.author | Partap, S. | en |
dc.contributor.author | Klimo, P. | en |
dc.contributor.author | Harreld, J. H. | en |
dc.contributor.author | Tinkle, C. L. | en |
dc.contributor.author | Indelicato, D. J. | en |
dc.date.accessioned | 2022-11-07T23:47:27Z | - |
dc.date.available | 2022-11-07T23:47:27Z | - |
dc.date.issued | 2018 | en |
dc.identifier.citation | 36, (15), 2018 | en |
dc.identifier.other | RIS | en |
dc.identifier.uri | http://dora.health.qld.gov.au/qldresearchjspui/handle/1/3917 | - |
dc.description.abstract | Background: Retrospective reports of poor outcomes of ependymoma (EPN) subgroups, posterior fossa A (PF-EPN-A) and supratentorial C11orf95-RELA (ST-EPN-RELA), need confirmation in prospective trials. Methods: Fifty-four children (median age 1.6 y, range 0.4-3.1) with newly diagnosed EPN (WHO grade II/ III) were treated (2008-2016) with maximal safe surgical resection + chemotherapy (high-dose methotrexate, vincristine, cisplatin and cyclophosphamide), consolidation using focal conformal radiation therapy (RT) to 54Gy (M0) or additional cyclophosphamide/topotecan with no RT (M+) and 6 months of oral maintenance chemotherapy. DNA methylation was performed using Infinium MethylationEPIC BeadChip and profiled on the DKFZ MN2.0 classifier. Fluorescent in-situ hybridization was used to determine tumor 1q status. Results: No participant had imaging evidence of metastatic disease at diagnosis (M0 = 40, M1 = 1, CSF not obtained = 13). At a median follow-up of 3.6 y (range, 1.0-9.3), 49 patients (91%) were alive with a 4-y PFS = 77.0%±7.5% and OS = 91.3%±5.2%. There was no significant difference in outcomes by subgroup [4-y PFS: PF-EPN-A (n = 42), 74.3%±8.2%; ST-EPN-RELA (n = 8), 80%±20.7%; ST-EPN-YAP (n = 4), 100%, p = 0.42]. Five patients with PF-EPN-A had 1q gain but no difference in outcome (p = 0.59 for OS and p = 0.15 for PFS). For 6 patients with subtotal resection (STR) prior to RT, outcome was inferior to those of the 48 with gross-total or near-total resection (4-y PFS = 27.8%±16.7% vs 81.7% ±7.3%, p = 0.047). Fourteen patients experienced progression at a median time of 28 mos (range, 1.7m-7.3 y). Recurrence was distant (n = 7), local (n = 6), or combined (n = 1), 3/14 recurred at 6 (n = 2) and 7 years post diagnosis. Conclusions: In a uniformly treated, prospective EPN cohort, we found no significant difference in PFS or OS by molecular subgroup; however, the number of ST-EPNRELA was small. In our data, 1q was not associated with outcome in PF-EPN-A, though only 5 subjects had 1q gain. Patients with STR prior to RT had inferior outcomes. Close surveillance and follow-up beyond 5 years is warranted due to risk of metastasis and late progression.L6259763632019-01-22 <br /> | en |
dc.language.iso | en | en |
dc.relation.ispartof | Journal of Clinical Oncology | en |
dc.title | Outcomes for young children with molecularly defined ependymoma treated on the multi-institutional SJYC07 clinical trial | en |
dc.type | Article | en |
dc.identifier.doi | 10.1200/JCO.2018.36.15_suppl.10548 | en |
dc.subject.keywords | child | en |
dc.subject.keywords | classifier | en |
dc.subject.keywords | clinical article | en |
dc.subject.keywords | cohort analysis | en |
dc.subject.keywords | conference abstract | en |
dc.subject.keywords | conformal radiotherapy | en |
dc.subject.keywords | controlled study | en |
dc.subject.keywords | DNA methylation | en |
dc.subject.keywords | drug megadose | en |
dc.subject.keywords | drug therapy | en |
dc.subject.keywords | ependymoma | en |
dc.subject.keywords | female | en |
dc.subject.keywords | fluorescence in situ hybridization | en |
dc.subject.keywords | follow up | en |
dc.subject.keywords | human | en |
dc.subject.keywords | transcription factor RelA | en |
dc.subject.keywords | maintenance chemotherapy | en |
dc.subject.keywords | male | en |
dc.subject.keywords | metastasis | en |
dc.subject.keywords | multicenter study | en |
dc.subject.keywords | prospective study | en |
dc.subject.keywords | radiotherapy | en |
dc.subject.keywords | relapse | en |
dc.subject.keywords | surgery | en |
dc.subject.keywords | topotecan | en |
dc.subject.keywords | methotrexate | en |
dc.subject.keywords | endogenous compound | en |
dc.subject.keywords | cisplatincyclophosphamide | en |
dc.subject.keywords | infant | en |
dc.subject.keywords | vincristine | en |
dc.subject.keywords | cancer radiotherapy | en |
dc.subject.keywords | cancer recurrence | en |
dc.subject.keywords | cancer surgery | en |
dc.relation.url | https://www.embase.com/search/results?subaction=viewrecord&id=L625976363&from=exporthttp://dx.doi.org/10.1200/JCO.2018.36.15_suppl.10548 | | en |
dc.identifier.risid | 2059 | en |
item.grantfulltext | none | - |
item.openairetype | Article | - |
item.fulltext | No Fulltext | - |
item.languageiso639-1 | en | - |
item.openairecristype | http://purl.org/coar/resource_type/c_18cf | - |
item.cerifentitytype | Publications | - |
Appears in Sites: | Children's Health Queensland Publications |
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