[Ewing sarcoma of the bone. Multidisciplinary approach and oncological results in 88 patients].

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Abstract

[Indexed for MEDLINE] 20. Br J Cancer. 2026 Jan;134(2):279-288. doi: 10.1038/s41416-025-03263-3. Epub 2025 Nov 17. Interest of a sequential multimodal approach for the treatment of newly diagnosed patients with multimetastatic Ewing sarcoma: results of the French prospective CombinaiR3 phase II trial. Laurence V(1), Jehanno N(2), Dureau S(3), Mous L(4), Claude L(5), Ballet S(6), Pierron G(6), Gaspar N(7), Brahmi M(8), Valentin T(9), Revon-Rivière G(10), Lervat C(11), Probert J(12), Entz-Werle N(13), Mansuy L(14), Plantaz D(15), Rios M(16), Saumet L(17), Vérité C(18), Castex MP(19), Thebaud E(20), Cassou-Mounat T(21), Plissonnier AS(22), Dieppedale J(3), Delattre O(23), Legrier ME(22), Marec-Bérard P(24), Gouin F(25), Berlanga P(7), Cordero C(1), Surdez D(4), Corradini N(26). Author information: (1)Adolescents and Young Adults Unit, Medical Oncology and SIREDO (Care, Innovation and Research for Children, Adolescents, and Young Adults with Cancer) Departments, Curie Institute, Paris, France. (2)Department of Nuclear Medicine, Curie Institute, Paris, France. (3)Department of Biometry, Curie Institute, Saint Cloud, France. (4)Balgrist University Hospital, Faculty of Medicine, University of Zurich (UZH), Zurich, Switzerland. (5)Department of Radiation Oncology, Centre Léon Bérard, Lyon, France. (6)Genetics Department Curie Institute & PSL Research University, Paris, France. (7)Department of Oncology for Children and Adolescents, Gustave Roussy Institute, Villejuif, France. (8)Department of Medical Oncology, Centre Léon Bérard, Lyon, France. (9)Department of Medical Oncology, Institut Claudius Regaud, Institut Universitaire du Cancer de Toulouse - Oncopole, Toulouse, France. (10)Department of Pediatric Hematology, Immunology and Oncology, APHM - La Timone Children's Hospital, Marseille, France. (11)Department of Pediatric and AYA Oncology, Centre Oscar Lambret, Lille, France. (12)Department of Pediatric Onco-hematology, University Hospital of Rennes, Rennes, France. (13)Department of Pediatric Onco-hematology, University Hospitals of Strasbourg, Strasbourg, France. (14)Department of Pediatric Hematology and Oncology, Children's University Hospital, Nancy, France. (15)Department of Pediatric Hematology and Oncology, University Hospital of Grenoble, Grenoble, France. (16)Department of Medical Oncology, Institut de Cancérologie de Lorraine-Alexis Vautrin, Vandoeuvre-lès-Nancy, France. (17)Department of Pediatric Onco-Hematology, University Hospital of Montpellier, Montpellier, Languedoc-Roussillon, France. (18)Department of Pediatric and Adolescent Hematology and Oncology, Pellegrin Hospital, Bordeaux, France. (19)Pediatric Oncology Immunology Hematology Unit, Children's University Hospital - Toulouse University Hospital, Toulouse, France. (20)Department of Pediatric Oncology, Hôpital Mère-Enfant, Nantes, France. (21)Nuclear Medicine Department, Institut Claudius Regaud, Institut Universitaire du Cancer Toulouse Oncopole, Toulouse, France. (22)DREH Pôle Promotion, Institut Curie, Saint-Cloud, France. (23)INSERM U830, Diversity and Plasticity of Childhood Tumors Lab, SIREDO Oncology Center, Curie Institute Research Center, PSL Research University, Paris, France. (24)Department of Pediatric Oncology, Institute for Paediatric Haematology and Oncology, Centre Léon Bérard, Lyon, France. (25)Department of Surgical Oncology, Centre Léon Bérard, Lyon, France. (26)Department of Pediatric Oncology, Institute for Paediatric Haematology and Oncology, Centre Léon Bérard, Lyon, France. nadege.corradini@ihope.fr. BACKGROUND: Ewing sarcoma (ES) is a rare tumour with metastatic spread in ~25% of cases at diagnosis. Extrapulmonary disseminated disease defines very high-risk (VHR) patients, with frequent relapse and poor overall survival (OS, ~30%). METHODS: The phase II CombinaiR3 trial (NCT03011528) enroled 45 VHR ES patients across 15 French centres (2017-2021) to evaluate a strategy combining dose-dense induction chemotherapy, high-dose consolidation, and prolonged maintenance therapy. The primary endpoint was median event-free survival (EFS). Exploratory endpoints included full-body-fluorine-18-fluorodeoxyglucose-positron emission tomography/computed tomography (PET/CT) and circulating tumour DNA (ctDNA) detection during treatment. RESULTS: Among 42 analysed patients (median age 14 years, range 6-47), 29 had a primary tumour volume ≥200 ml, and 35 presented with bone ± bone marrow metastatic lesions, 18 exhibiting more than 5 bone lesions. At 48-month follow-up, 18- and 36-month EFS rates were 63.4% and 53.7%, respectively, with 3-year OS at 65.5%. Toxicity was as expected, with no treatment-related deaths or maintenance therapy discontinuations due to toxicity. PET/CT and ctDNA monitoring showed strong correlation at diagnosis and relapse. DISCUSSION: This study supports the proposed experimental strategy as a first-line option for selected VHR ES patients, warranting integration into international therapeutic discussions. © 2025. The Author(s), under exclusive licence to Springer Nature Limited. DOI: 10.1038/s41416-025-03263-3 PMCID: PMC12820225