{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,17]],"date-time":"2025-12-17T12:58:49Z","timestamp":1765976329311,"version":"3.48.0"},"reference-count":39,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2025,9,19]],"date-time":"2025-09-19T00:00:00Z","timestamp":1758240000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005972","name":"Deutsche Krebshilfe","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005972","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100007311","name":"Deutsche Kinderkrebsstiftung","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007311","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp;amp; Cancer"],"published-print":{"date-parts":[[2025,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Extracranial malignant rhabdoid tumors (eMRT) are rare, highly aggressive pediatric neoplasms. While the liver is a relatively common anatomic site of presentation, the clinical course of patients with hepatic eMRT (eMRT\u2010L) is not well described.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      We retrospectively analyzed 30 children affected by eMRT\u2010L treated on a consensus regimen provided by the European Rhabdoid Registry (EU\u2010RHAB). Clinical characteristics, radiology features according to the Pre\u2010Treatment Extent of Tumor (PRETEXT) system, treatment details, and outcome were assessed. We employed patients with rhabdoid tumors of the kidney (RTK;\n                      <jats:italic>n<\/jats:italic>\n                      = 30) and other eMRT (\n                      <jats:italic>n<\/jats:italic>\n                      = 60) as controls.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Median age at diagnosis was 8\u00a0months (range: 0\u201353\u00a0months), 16 of 30 patients (55%) presented with metastatic disease. R0 resection was achieved in seven patients (23%). Most tumors showed PRETEXT Stage\n                      <jats:bold>\u2265<\/jats:bold>\n                      3 (66%) and frequently exhibited PRETEXT annotation factors. One\u2010year overall and event\u2010free survivals were both 17% (95% confidence interval: 7.5\u201337). Compared to RTK and other eMRT, patients with eMRT\u2010L had significantly inferior outcomes (hazard ratios 2.47 and 4.39, respectively). Complete resection and absence of metastases were associated with improved survival. Consolidation therapies (i.e., radiotherapy or high\u2010dose chemotherapy) were only rarely used.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>EMRT\u2010L represents a distinct high\u2010risk subgroup within the eMRT spectrum, characterized by inferior survival despite standardized multimodal therapy. Current treatment approaches demonstrate limited efficacy. Our results highlight the urgent need for prospective, collaborative studies to refine risk stratification and to evaluate novel treatment options.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/pbc.32062","type":"journal-article","created":{"date-parts":[[2025,9,20]],"date-time":"2025-09-20T06:40:28Z","timestamp":1758350428000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Malignant Rhabdoid Tumors of the Liver Are Associated With Inferior Outcomes Compared to Other Extracranial Rhabdoid Tumors"],"prefix":"10.1002","volume":"72","author":[{"ORCID":"https:\/\/orcid.org\/0009-0003-2308-477X","authenticated-orcid":false,"given":"Sebastian","family":"B\u00fchner","sequence":"first","affiliation":[{"name":"Pediatrics and Adolescent Medicine Swabian Children's Cancer Center University Hospital Augsburg  Augsburg Germany"},{"name":"Bavarian Cancer Research Center (BZKF)  Augsburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katharina","family":"Gastberger","sequence":"additional","affiliation":[{"name":"Pediatrics and Adolescent Medicine Swabian Children's Cancer Center University Hospital Augsburg  Augsburg Germany"},{"name":"Bavarian Cancer Research Center (BZKF)  Augsburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7952-1380","authenticated-orcid":false,"given":"Stefanie","family":"T\u00fcchert\u2010Knoll","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Interventional Radiology University Hospital Augsburg  Augsburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7488-6885","authenticated-orcid":false,"given":"Victoria E.","family":"Fincke","sequence":"additional","affiliation":[{"name":"Pediatrics and Adolescent Medicine Swabian Children's Cancer Center University Hospital Augsburg  Augsburg Germany"},{"name":"Bavarian Cancer Research Center (BZKF)  Augsburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pascal D.","family":"Johann","sequence":"additional","affiliation":[{"name":"Pediatrics and Adolescent Medicine Swabian Children's Cancer Center University Hospital Augsburg  Augsburg Germany"},{"name":"Bavarian Cancer Research Center (BZKF)  Augsburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patrick","family":"Melchior","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology Saarland University Hospital  Homburg Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Margarita","family":"Teleshova","sequence":"additional","affiliation":[{"name":"Dmitry Rogachev National Medical Research Center of Pediatric Hematology Oncology and Immunology  Moscow 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