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Eligible patients were evaluated for patient, tumor, and treatment related variables, and outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>One-hundred thirty-two eligible patients were identified (median age 41.7 (range: 10.3\u201374.6) years; males 58%; preceding malignancies 11%; tumor-sites extremities 86%, trunk 12%, head &amp; neck 2%). Relative tumor size was\u2009&lt;\u20091\/3 of the involved bone in 83% of evaluable cases, pathologic fractures were present in 14% (limb-primaries only), and primary distant metastases in 6%. All patients received chemotherapy and 96% surgery for their primary (81% limb-salvage for extremity lesions). The response rate to preoperative chemotherapy was 38%. After a median follow-up of 3.9 (1\u00a0day\u201334.1) years for event-free and 5.2 (0.2\u201334.1) years for overall survival, the 5\u00a0year event-free and overall survival probabilities were 63% (standard error: 5%) and 70% (4%), respectively. Younger patient age, an extremity tumor, and localized disease predicted superior outcomes, pathologic fractures and limb-salvage surgery worse. The extent of tumor response to pre-operative chemotherapy seemed to have no impact on prognosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>UPSB generally affects considerably older persons than does the more frequent osteosarcoma. Despite a lower response rate to preoperative chemotherapy, its prognosis is at least comparable, if not better. Several prognostic factors impacting outcome could be defined. These results provide a benchmark for this rare disease.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00432-025-06405-5","type":"journal-article","created":{"date-parts":[[2026,1,20]],"date-time":"2026-01-20T16:43:15Z","timestamp":1768927395000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Undifferentiated pleomorphic sarcoma of bone (UPSB) treated in the German-speaking countries. 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Stefan Bielack reports consulting fees from SERB SAS (Advisory Board, payment to himself), consulting fees from Atheneum Partners GmbH (payment to himself), and consulting fees from Medicys Healthcare Fieldwork Experts (payment to himself); and payment for expert testimony from Zschimmer & Schwarz Mohsdorf GmbH & Co. KG (payment to himself). Stefanie Hecker-Nolting reports support for the present manuscript from F\u00f6rderkreis Krebskranke Kinder e.V. Stuttgart, Germany (Charity foundation, funding); grants or contracts from Deutsche Kinderkrebsstiftung, Bonn, Germany (Charity foundation, grant for COSS); leadership role in FOSTER (Fight Osteosarcoma through European Research), member of Executive Committee (EC), Lead WP3 (unpaid); leadership role in COSS (Cooperative Osteosarcoma Study Group) (head of study group); and leadership role in HIBISCUS (member of EC) Thekla von Kalle reports leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid for GPR\u2014German Speaking Society of Paediatric Radiology (President, no payment), and ESPR\u2014European Soc. of Paediatric radiology (Member of Oncology task force, no payment). Thomas K\u00fchne reports support for the present manuscript from IPSEN (honoraria). Peter Reichardt reports consulting fees from Bayer (to institution), GSK (to institution), PharmaMar (to institution), and Boehringer Ingelheim (to institution); payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from PharmaMar (to institution) and Boehringer Ingelheim (to institution); and leadership or fiduciary role in other board, society, committee or advocacy group, paid or unpaid as chairman of the German Sarcoma Foundation. Reinhard Windhager reports consulting fees from Johnson & Johnson Medical Limited and consulting fees from Stryker European Operations Limited; and other financial or non-financial interests from De Puy Synthes (Product Development Agreement with Medical University Vienna), other financial or non-financial interests from Johnson & Johnson Medical Limited (Life Case Obsevation Agreement with Medical University Vienna), and other financial or non-financial interests from Johnson & Johnson Medical Limited (Educational Agreement: MedUni with Medical University Vienna). Daniel Baumhoer, Claudia Blattmann, Godehard Friedel, Birgit Fr\u00f6hlich, Semi Ben Harrabi, Leo Kager Torsten Kessler, Matthias Kevric, Antonia Knoll, and Benjamin Sorg report no conflicts of interest to disclose.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"The studies and registries which form the basis of this work were approved by Ethik-Kommission bei der \u00c4rztekammer Hamburg nos. 500, 1147; Ethikkommission der \u00c4rztekammer Westfalen-Lippe und der Westf\u00e4lischen-Wilhelms Universit\u00e4t nos. 182\/98 Biel2, 4IV Bie 2, 4 I Bielack, 5\u00a0V. Bielack; and Ethik-Kommission an der Medizinischen Fakult\u00e4t der Eberhard-Karls-Universit\u00e4t und am Universit\u00e4tsklinikum T\u00fcbingen no. 5\u00a0V Bielack.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval"}},{"value":"Informed consent was obtained from all patients and\/or legal guardians, whichever appropriate, before enrolment into the COSS-database.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}},{"value":"Not applicable.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Clinical trial registration"}},{"value":"Not applicable.","order":6,"name":"Ethics","group":{"name":"EthicsHeading","label":"Permission to reproduce material from other sources"}}],"article-number":"40"}}