Please use this identifier to cite or link to this item: https://dora.health.qld.gov.au/qldresearchjspui/handle/1/3917
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dc.contributor.authorEllison, D. W.en
dc.contributor.authorBoop, F. A.en
dc.contributor.authorMerchant, T. E.en
dc.contributor.authorGajjar, A. J.en
dc.contributor.authorUpadhyaya, S.en
dc.contributor.authorRobinson, G. W.en
dc.contributor.authorOrr, B.en
dc.contributor.authorOnar-Thomas, A.en
dc.contributor.authorBillups, C. A.en
dc.contributor.authorArmstrong, G. T.en
dc.contributor.authorSadighi, Z. S.en
dc.contributor.authorHassall, T.en
dc.contributor.authorBowers, D. C.en
dc.contributor.authorBendel, A. E.en
dc.contributor.authorCrawford, J. R.en
dc.contributor.authorPartap, S.en
dc.contributor.authorKlimo, P.en
dc.contributor.authorHarreld, J. H.en
dc.contributor.authorTinkle, C. L.en
dc.contributor.authorIndelicato, D. J.en
dc.date.accessioned2022-11-07T23:47:27Z-
dc.date.available2022-11-07T23:47:27Z-
dc.date.issued2018en
dc.identifier.citation36, (15), 2018en
dc.identifier.otherRISen
dc.identifier.urihttp://dora.health.qld.gov.au/qldresearchjspui/handle/1/3917-
dc.description.abstractBackground: 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.isoenen
dc.relation.ispartofJournal of Clinical Oncologyen
dc.titleOutcomes for young children with molecularly defined ependymoma treated on the multi-institutional SJYC07 clinical trialen
dc.typeArticleen
dc.identifier.doi10.1200/JCO.2018.36.15_suppl.10548en
dc.subject.keywordschilden
dc.subject.keywordsclassifieren
dc.subject.keywordsclinical articleen
dc.subject.keywordscohort analysisen
dc.subject.keywordsconference abstracten
dc.subject.keywordsconformal radiotherapyen
dc.subject.keywordscontrolled studyen
dc.subject.keywordsDNA methylationen
dc.subject.keywordsdrug megadoseen
dc.subject.keywordsdrug therapyen
dc.subject.keywordsependymomaen
dc.subject.keywordsfemaleen
dc.subject.keywordsfluorescence in situ hybridizationen
dc.subject.keywordsfollow upen
dc.subject.keywordshumanen
dc.subject.keywordstranscription factor RelAen
dc.subject.keywordsmaintenance chemotherapyen
dc.subject.keywordsmaleen
dc.subject.keywordsmetastasisen
dc.subject.keywordsmulticenter studyen
dc.subject.keywordsprospective studyen
dc.subject.keywordsradiotherapyen
dc.subject.keywordsrelapseen
dc.subject.keywordssurgeryen
dc.subject.keywordstopotecanen
dc.subject.keywordsmethotrexateen
dc.subject.keywordsendogenous compounden
dc.subject.keywordscisplatincyclophosphamideen
dc.subject.keywordsinfanten
dc.subject.keywordsvincristineen
dc.subject.keywordscancer radiotherapyen
dc.subject.keywordscancer recurrenceen
dc.subject.keywordscancer surgeryen
dc.relation.urlhttps://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.risid2059en
item.grantfulltextnone-
item.openairetypeArticle-
item.fulltextNo Fulltext-
item.languageiso639-1en-
item.openairecristypehttp://purl.org/coar/resource_type/c_18cf-
item.cerifentitytypePublications-
Appears in Sites:Children's Health Queensland Publications
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