{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,10]],"date-time":"2026-11-10T04:03:14Z","timestamp":1794283394686,"version":"4.9.0"},"reference-count":36,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2019,4,12]],"date-time":"2019-04-12T00:00:00Z","timestamp":1555027200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"National Institutes of Health and the American Lebanese Syrian Associated Charities"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,10,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>This report documents the clinical characteristics, molecular grouping, and outcome of young children with ependymoma treated prospectively on a clinical trial.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>Fifty-four children (aged \u22643 y) with newly diagnosed ependymoma were treated on the St Jude Young Children 07 (SJYC07) trial with maximal safe surgical resection, 4 cycles of systemic chemotherapy, consolidation therapy using focal conformal radiation therapy (RT) (5-mm clinical target volume), and 6 months of oral maintenance chemotherapy. Molecular groups were determined by tumor DNA methylation using Infinium Methylation EPIC BeadChip and profiled on the German Cancer Research Center\/Molecular Neuropathology 2.0 classifier.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>One of the 54 study patients had metastases (cerebrospinal fluid positive) at diagnosis. Gross or near-total resection was achieved in 48 (89%) patients prior to RT. At a median follow-up of 4.4 years (range, 0.2\u201310.3 y), 4-year progression-free survival (PFS) was 75.1% \u00b1 7.2%, and overall survival was 92.6% \u00b1 4.4%. The molecular groups showed no significant difference in PFS (4-year estimates: posterior fossa ependymoma group A [PF-EPN-A; 42\/54], 71.2% \u00b1 8.3%; supratentorial ependymoma positive for v-rel avian reticuloendotheliosis viral oncogene homolog A [ST-EPN-RELA; 8\/54], 83.3% \u00b1 17.0%; and supratentorial ependymoma positive for Yes-associated protein [4\/54], 100%, P = 0.22). Subtotal resection prior to RT was associated with an inferior PFS compared with gross or near-total resection (4-year PFS: 41.7% \u00b1 22.5% vs 79.0% \u00b1 7.1%, P = 0.024), as was PF-EPN-A group with 1q gain (P = 0.05). Histopathologic grading was not associated with outcomes (classic vs anaplastic; P = 0.89).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>In this prospectively treated cohort of young children with ependymoma, ST-EPN-RELA tumors had a more favorable outcome than reported from retrospective data. Histologic grade did not impact outcome. PF-EPN-A with 1q gain and subtotal resection were associated with inferior outcomes.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/neuonc\/noz069","type":"journal-article","created":{"date-parts":[[2019,4,8]],"date-time":"2019-04-08T15:24:41Z","timestamp":1554737081000},"page":"1319-1330","source":"Crossref","is-referenced-by-count":95,"title":["Molecular grouping and outcomes of young children with newly diagnosed ependymoma treated on the multi-institutional SJYC07 trial"],"prefix":"10.1093","volume":"21","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6101-149X","authenticated-orcid":false,"given":"Santhosh A","family":"Upadhyaya","sequence":"first","affiliation":[{"name":"Department of Oncology, St Jude Children\u2019s Research Hospital, Memphis, Tennessee, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giles W","family":"Robinson","sequence":"additional","affiliation":[{"name":"Department of Oncology, St Jude Children\u2019s Research Hospital, Memphis, Tennessee, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arzu","family":"Onar-Thomas","sequence":"additional","affiliation":[{"name":"Department of Biostatistics, St Jude Children\u2019s Research Hospital, Memphis, Tennessee, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brent A","family":"Orr","sequence":"additional","affiliation":[{"name":"Department of Pathology, St Jude Children\u2019s Research Hospital, Memphis, Tennessee, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine A","family":"Billups","sequence":"additional","affiliation":[{"name":"Department of Oncology, St Jude Children\u2019s Research Hospital, Memphis, Tennessee, USA"},{"name":"Department of Biostatistics, St Jude Children\u2019s Research Hospital, Memphis, Tennessee, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel C","family":"Bowers","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics and Neurological Surgery, University of Texas Southwestern Medical School\/Children\u2019s Health, Dallas, Texas, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne E","family":"Bendel","sequence":"additional","affiliation":[{"name":"Department of Hematology Oncology, Children\u2019s Hospitals and Clinics of Minnesota, Minneapolis, Minnesota, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tim","family":"Hassall","sequence":"additional","affiliation":[{"name":"Department of Medicine, Queensland Children\u2019s Hospital, South Brisbane, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John R","family":"Crawford","sequence":"additional","affiliation":[{"name":"Department of Neurosciences and Pediatrics, University of California San Diego and Rady Childrens Hospital, San Diego, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonia","family":"Partap","sequence":"additional","affiliation":[{"name":"Department of Neurology & Division of Child Neurology, Stanford University, Palo Alto, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul G","family":"Fisher","sequence":"additional","affiliation":[{"name":"Department of Neurology & Division of Child Neurology, Stanford University, Palo Alto, California, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruth G","family":"Tatevossian","sequence":"additional","affiliation":[{"name":"Department of Pathology, St Jude Children\u2019s Research Hospital, Memphis, Tennessee, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tiffany","family":"Seah","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Cambridge, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ibrahim A","family":"Qaddoumi","sequence":"additional","affiliation":[{"name":"Department of Oncology, St Jude Children\u2019s Research Hospital, Memphis, Tennessee, USA"},{"name":"Department of Global Pediatric Medicine, St Jude Children\u2019s Research Hospital, Memphis, Tennessee, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Vinitsky","sequence":"additional","affiliation":[{"name":"Department of Oncology, St Jude Children\u2019s Research Hospital, Memphis, Tennessee, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregory T","family":"Armstrong","sequence":"additional","affiliation":[{"name":"Department of Oncology, St Jude Children\u2019s Research Hospital, Memphis, Tennessee, USA"},{"name":"Department of Epidemiology and Cancer Control, St Jude Children\u2019s Research Hospital, Memphis, Tennessee, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noah D","family":"Sabin","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Imaging, St Jude Children\u2019s Research Hospital, Memphis, Tennessee, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher L","family":"Tinkle","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology, St Jude Children\u2019s Research Hospital, Memphis, Tennessee, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Klimo","sequence":"additional","affiliation":[{"name":"Department of Surgery, St Jude Children\u2019s Research Hospital, Memphis, Tennessee, USA"},{"name":"Semmes-Murphey Clinic, Memphis, Tennessee, USA"},{"name":"Department of Neurosurgery, University of Tennessee and Le Bonheur Children\u2019s Hospital, Memphis, Tennessee, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danny J","family":"Indelicato","sequence":"additional","affiliation":[{"name":"Department of Radiation 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