{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T10:35:55Z","timestamp":1777890955593,"version":"3.51.4"},"reference-count":25,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,2,20]],"date-time":"2026-02-20T00:00:00Z","timestamp":1771545600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,2,20]],"date-time":"2026-02-20T00:00:00Z","timestamp":1771545600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100002002","name":"Cancer Research Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100018762","name":"Seattle Children's Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100018762","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100003287","name":"Rally Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100003287","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100016916","name":"Royal Marsden Cancer Charity","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100016916","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005954","name":"Children\u2019s Cancer Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005954","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer Medicine"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>We evaluated the survival rate\/survivor characteristics following first progression\/relapse of metastatic rhabdomyosarcoma (M1 RMS), using pooled European and US collaborative group data from the INternational Soft Tissue saRcoma ConsorTium (INSTRuCT).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patients with first diagnosis of M1 RMS aged 0\u201340\u2009years were identified within the INSTRuCT database (Upfront Cohort; UC). The First Event Cohort (FEC) included UC patients with first event of disease progression\/relapse. Clinical features and survival of FEC patients were described.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      UC included 1095 eligible M1 RMS patients. 5\u2010year Overall and Event Free Survival were 32.0% (95% Confidence Interval (CI) 29.2\u201334.9) and 27.5% (95% CI 24.8\u201330.2) respectively. Median time to event was 13.9\u2009months (range 1\u2009day\u2010172.6\u2009months). Among UC patients, 727 with first event of progression\/relapse were included in FEC. 3\u2010year Overall Survival for FEC from first event was 8.0% (95% CI 6.1\u201310.2). Thirty\u2010four (4.7%) FEC patients were alive with &gt;\u20093\u2009years follow up (\u201cdisease free\u201d) and 16 (2.2%) with\n                      <jats:styled-content>&lt;<\/jats:styled-content>\n                      3\u2009years follow up. FEC patients alive &gt;\u20093\u2009years were significantly more likely than deceased FEC patients to have: younger age (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.0031); no locoregional lymph node involvement (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.0013); fewer metastatic sites (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.006); no bone and\/or bone marrow disease (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001 for each); lower Oberlin scores (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.0001); time to first event &gt;\u200918\u2009months (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.0001). Univariate and multivariable analyses conducted in FEC to investigate factors impacting OS showed that Oberlin score \u2265\u20092 (Hazard Ratio (HR) 1.295, 95% Confidence Limits (CL) 1.07\u20131.57,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.0074) and involvement of loco\u2010regional lymph nodes at diagnosis (HR 1.28, 95% CL 1.08\u20131.52,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.0053) were associated with worse outcome.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Outcomes following first progression\/relapse of M1 RMS are dismal. Survivors had fewer adverse prognostic features at first presentation and later first events. Further work is required to predict survivors of first relapse more reliably.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/cam4.71524","type":"journal-article","created":{"date-parts":[[2026,2,21]],"date-time":"2026-02-21T05:15:32Z","timestamp":1771650932000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Children and Young People With First Relapse or Progression of Upfront Metastatic Rhabdomyosarcoma: An Analysis of Clinical Features and Outcomes From the\n                    <scp>INternational<\/scp>\n                    Soft Tissue\n                    <scp>saRcoma ConsorTium<\/scp>\n                    (\n                    <scp>INSTRuCT<\/scp>\n                    )"],"prefix":"10.1002","volume":"15","author":[{"given":"Ajla T.","family":"Wasti","sequence":"first","affiliation":[{"name":"Institute of Cancer Research  Sutton UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gianni","family":"Bisogno","sequence":"additional","affiliation":[{"name":"Department of Women and Children's Health University of Padova  Padova Italy"},{"name":"Pediatric Hematology Oncology Division University Hospital of Padova  Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beatrice","family":"Coppadoro","sequence":"additional","affiliation":[{"name":"Department of Women and Children's Health University of Padova  Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ilaria","family":"Zanetti","sequence":"additional","affiliation":[{"name":"Pediatric Hematology Oncology Division University Hospital of Padova  Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin","family":"Ebinger","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology and Oncology University Children's Hospital  T\u00fcbingen Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3472-9679","authenticated-orcid":false,"given":"Amadeus T.","family":"Heinz","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology and Oncology University Children's Hospital  T\u00fcbingen Germany"},{"name":"Stuttgart Cancer Center, Zentrum f\u00fcr Kinder\u2010, Jugend\u2010 und Frauenmedizin (Olgahospital), P\u00e4diatrie 5 (P\u00e4diatrische Onkologie, H\u00e4matologie, Immunologie) Klinikum der Landeshauptstadt Stuttgart  Stuttgart Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Henry C.","family":"Mandeville","sequence":"additional","affiliation":[{"name":"Institute of Cancer Research  Sutton UK"},{"name":"Children and Young People's Unit Royal Marsden Hospital  Sutton UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rita","family":"Alaggio","sequence":"additional","affiliation":[{"name":"Istituto di Ricovero e Cura a Carattere Scientifico Bambino Ges\u00f9 Children's Hospital  Rome Italy"},{"name":"Department of Medico\u2010Surgical Sciences and Biotechnologies Universit\u00e0 Sapienza  Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michela","family":"Casanova","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Istituto Nazionale dei Tumori  Milan Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sheila E. J. Terwisscha","family":"van Scheltinga","sequence":"additional","affiliation":[{"name":"Princess M\u00e1xima Center for Pediatric Oncology  Utrecht the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rick R.","family":"van Rijn","sequence":"additional","affiliation":[{"name":"Department of Radiology and Nuclear Medicine University of Amsterdam, Amsterdam UMC  Amsterdam the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0296-5207","authenticated-orcid":false,"given":"Veronique","family":"Minard\u2010Colin","sequence":"additional","affiliation":[{"name":"Department of Pediatric and Adolescent Oncology, INSERM U1015, Gustave Roussy Universit\u00e9 Paris\u2010Saclay  Villejuif France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2520-139X","authenticated-orcid":false,"given":"Daniel","family":"Orbach","sequence":"additional","affiliation":[{"name":"SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults With Cancer) PSL University, Institut Curie  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Natalie B.","family":"Collins","sequence":"additional","affiliation":[{"name":"Dana\u2010Farber\/Boston Children's Cancer and Blood Disorders Center Harvard Medical School  Boston Massachusetts USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wei","family":"Xue","sequence":"additional","affiliation":[{"name":"Department of Biostatistics University of Florida  Gainesville Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rajkumar","family":"Venkatramani","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Hematology Oncology Baylor College of Medicine  Houston Texas USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johannes H. M.","family":"Merks","sequence":"additional","affiliation":[{"name":"Princess M\u00e1xima Center for Pediatric Oncology  Utrecht the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3479-7997","authenticated-orcid":false,"given":"Julia C.","family":"Chisholm","sequence":"additional","affiliation":[{"name":"Institute of Cancer Research  Sutton UK"},{"name":"Children and Young People's Unit Royal Marsden Hospital  Sutton UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2026,2,20]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2007.14.7207"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.1999.17.11.3487"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.21.02981"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.30707"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.26645"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/cam4.2504"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.21138"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2007.12.2382"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2010.32.1984"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.29924"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.31770"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2006.07.1720"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2015.63.4048"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2009.22.3768"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2004.04.083"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21494"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.3390\/cancers15072050"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2010.02.051"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2009.08.019"},{"key":"e_1_2_11_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.31477"},{"key":"e_1_2_11_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21906"},{"key":"e_1_2_11_23_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2010.29.7390"},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.19.00576"},{"key":"e_1_2_11_25_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.21.00124"},{"key":"e_1_2_11_26_1","doi-asserted-by":"publisher","DOI":"10.3390\/cancers16050998"}],"container-title":["Cancer Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cam4.71524","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/cam4.71524","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cam4.71524","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T05:50:19Z","timestamp":1777614619000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/cam4.71524"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,20]]},"references-count":25,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2026,3]]}},"alternative-id":["10.1002\/cam4.71524"],"URL":"https:\/\/doi.org\/10.1002\/cam4.71524","archive":["Portico"],"relation":{},"ISSN":["2045-7634","2045-7634"],"issn-type":[{"value":"2045-7634","type":"print"},{"value":"2045-7634","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,20]]},"assertion":[{"value":"2025-05-31","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-12-23","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-02-20","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e71524"}}