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oai:recercat.cat:11351/124352025-10-24T10:40:01Zcom_2072_378070com_2072_378040col_2072_378092

Localized incompletely resected standard risk rhabdomyosarcoma in children and adolescents: Results from the European Paediatric Soft Tissue Sarcoma Study Group RMS 2005 trial Mandeville, Henry alaggio, rita Ben-Arush, Myriam Hladun Alvaro, Raquel Ramos Albiac, Monica Bisogno, Gianni Minard-Colin, Veronique Chargari, Cyrus Institut Català de la Salut [Mandeville HC] The Royal Marsden Hospital and The Institute of Cancer Research, Surrey, UK. [Bisogno G] Hematology Oncology Division, Department of Women’s and Children’s Health, University of Padova, Padova, Italy. [Minard‐Colin V] Department of Pediatric and Adolescent Oncology, INSERM U1015, Gustave Roussy, Université Paris‐Saclay, Villejuif, France. [Alaggio R] Pathology Unit, Department of Laboratories, Bambino Gesu Children's Hospital, IRCCS, Rome, Italy. [Ben‐Arush M] Ruth Rappaport Children's Hospital, Rambam Medical Center, Joan and Sanford Weill Pediatric Hematology Oncology and Bone Marrow Transplantation Division, Haifa, Israel. [Chargari C] Radiation Oncology, Gustave Roussy Cancer Campus, Villejuif, France. [Hladun Alvaro R] Servei d'Hematologia i Oncologia Pediàtriques, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Ramos Albiac M] Servei de Radiodiagnòstic, Vall d’Hebron Hospital Universitari, Barcelona, Spain Vall d'Hebron Barcelona Hospital Campus Músculs - Càncer - Quimioteràpia Tumors de parts toves Músculs - Càncer - Radioteràpia Infants Quimioteràpia combinada DISEASES::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Connective and Soft Tissue::Neoplasms, Muscle Tissue::Myosarcoma::Rhabdomyosarcoma Other subheadings::Other subheadings::Other subheadings::/drug therapy ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Therapeutics::Radiotherapy NAMED GROUPS::Persons::Age Groups::Child ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Therapeutics::Therapeutics::Drug Therapy::Antineoplastic Protocols::Therapeutics::Drug Therapy::Antineoplastic Combined Chemotherapy Protocols ENFERMEDADES::neoplasias::neoplasias por tipo histológico::neoplasias de tejido conjuntivo y de tejidos blandos::neoplasias de tejido muscular::miosarcoma::rabdomiosarcoma Otros calificadores::Otros calificadores::Otros calificadores::/farmacoterapia TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::terapéutica::radioterapia DENOMINACIONES DE GRUPOS::personas::Grupos de Edad::niño TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::terapéutica::terapéutica::farmacoterapia::protocolos antineoplásicos::terapéutica::farmacoterapia::protocolos de quimioterapia antineoplásica combinada Alkylator chemotherapy; Radiotherapy; Rhabdomyosarcoma Quimioterapia alquilante; Radioterapia; Rabdomiosarcoma Quimioteràpia alquilant; Radioteràpia; Rabdomiosarcoma Background The authors report the prospective evaluation of reduced dose alkylator chemotherapy combined with radiotherapy for European Pediatric Soft Tissue Sarcoma Study Group (EpSSG) standard risk nonalveolar rhabdomyosarcoma (NA-RMS). Patients and Methods Localized node negative Intergroup Rhabdomyosarcoma Study (IRS) II/III NA-RMS at favorable sites (subgroup C), <25 years old, received five cycles of ifosfamide, vincristine, and dactinomycin (IVA) chemotherapy (30 g/m2 ifosfamide) and four cycles of vincristine and dactinomycin (if receiving radiotherapy), or nine cycles of IVA (54 g/m2 ifosfamide) ± radiotherapy. Delayed primary tumor excision was considered for IRS III tumors. The primary end points were event-free survival (EFS) and overall survival (OS). Results From October 2005 to December 2016, 359 evaluable patients were recruited: orbit, 164 (45.7%); head and neck nonparameningeal, 77 (21.4%); and genitourinary non–bladder/prostate, 118 (32.9%). EFS and OS were 77.4% (95% confidence interval [CI], 72.5–81.6) and 93.5% (95% CI, 90.1–95.8), respectively. Lower dose alkylator chemotherapy and radiotherapy achieved 5-year OS of 93.7% but the difference with higher dose alkylator chemotherapy +/- radiotherapy was not significant (p = 0.8003). Adjuvant radiotherapy improved EFS with 5-year estimates of 84.7% versus 65.2% for nonirradiated (p < .0001), but not OS (p = .9298). Omitting radiotherapy for orbital tumors reduced OS (5-year was 87.1% vs. 97.3% for irradiated, p = .0257). Following R0 resection (n = 60), radiotherapy did not significantly improve EFS or OS. Conclusions Radiotherapy for local tumor control allows for reduction of cumulative dose of alkylators in EpSSG standard risk subgroup C RMS patients. The omission of radiotherapy did not affect OS in all patients except those with orbital RMS and was associated with inferior EFS. 2025-01-17T13:10:18Z 2025-01-17T13:10:18Z 2024-12-01 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Mandeville HC, Bisogno G, Minard-Colin V, Alaggio R, Ben-Arush M, Chargari C, et al. Localized incompletely resected standard risk rhabdomyosarcoma in children and adolescents: Results from the European Paediatric Soft Tissue Sarcoma Study Group RMS 2005 trial. Cancer. 2024 Dec 1;130(23):4071–84. 1097-0142 https://hdl.handle.net/11351/12435 10.1002/cncr.35497 39058728 001278087300001 http://hdl.handle.net/11351/12435 eng Cancer;130(23) https://doi.org/10.1002/cncr.35497 Attribution 4.0 International http://creativecommons.org/licenses/by/4.0/ info:eu-repo/semantics/openAccess application/pdf Wiley Scientia