2026-04-17T06:27:55Zhttps://recercat.cat/oai/request

oai:recercat.cat:11351/136812025-10-24T10:17:42Zcom_2072_378070com_2072_378040col_2072_378092

Local therapy for rhabdomyosarcoma of the bladder and/or prostate without nodal or metastatic spread during the European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS2005 study Smeulders, Naima Guérin, Florent Rogers, Timothy Terwisscha van Scheltinga, Sheila Guillén Burrieza, Gabriela Gaze, Mark De Corti, Federica Infants Bufeta - Càncer - Tractament Pròstata - Càncer - Tractament Braquiteràpia Avaluació de resultats (Assistència sanitària) DISEASES::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Connective and Soft Tissue::Neoplasms, Muscle Tissue::Myosarcoma::Rhabdomyosarcoma Other subheadings::Other subheadings::/therapy ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Therapeutics::Radiotherapy::Brachytherapy DISEASES::Neoplasms::Neoplasms by Site::Urogenital Neoplasms::Urologic Neoplasms::Urinary Bladder Neoplasms DISEASES::Neoplasms::Neoplasms by Site::Urogenital Neoplasms::Genital Neoplasms, Male::Prostatic Neoplasms NAMED GROUPS::Persons::Age Groups::Child ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Diagnosis::Prognosis::Treatment Outcome::Progression-Free Survival ENFERMEDADES::neoplasias::neoplasias por tipo histológico::neoplasias de tejido conjuntivo y de tejidos blandos::neoplasias de tejido muscular::miosarcoma::rabdomiosarcoma Otros calificadores::Otros calificadores::/terapia TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::terapéutica::radioterapia::braquiterapia ENFERMEDADES::neoplasias::neoplasias por localización::neoplasias urogenitales::neoplasias urológicas::neoplasias de la vejiga ENFERMEDADES::neoplasias::neoplasias por localización::neoplasias urogenitales::neoplasias de los genitales masculinos::neoplasias de la próstata DENOMINACIONES DE GRUPOS::personas::Grupos de Edad::niño TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::diagnóstico::pronóstico::resultado del tratamiento::supervivencia libre de progresión Rhabdomyosarcoma; Local therapy; Organ-depleting surgery Rabdomiosarcoma; Terapia local; Cirugía preservadora de órganos Rabdomiosarcoma; Teràpia local; Cirurgia preservadora d'òrgans Background During the EpSSG RMS2005 trial, organ-sparing surgery (OSS) with brachytherapy (BT) became the local therapy (LT) of choice for selected patients with bladder-prostate rhabdomyosarcoma (BP-RMS). We compare this LT technique with surgical resection and/or external-beam radiotherapy. Methods Patients with BP-RMS without nodal or metastatic spread enrolled in RMS2005 were categorized by their LT, differentiating OSS from organ-depleting surgery (ODS) and BT from external-beam radiotherapy (EBRT). Progressive disease, relapse or death were considered events for progression-free survival (PFS) and all deaths for overall survival (OS). Results The cohort comprised 176 patients, aged 10days-21.8years (median 2.5years). Median follow-up was 6.5years (22months-12.5years): 5year-PFS was 80.3 % (95 %CI:73.6–85.5 %); 5year-OS was 90.7 % (95 %CI:85.3–94.2 %). Patients selected for surgery alone or BT with/without OSS (BT+/-OSS) differed significantly in age, tumour size and location from those offered EBRT alone or any other surgery and radiotherapy. Nevertheless, 5year-PFS was similar for the LT groups. However, 5year-OS differed significantly, being highest in patients suitable for surgery alone (100 %; by ODS in 55 %) or BT+ /-OSS (98.1 %; 95 %CI:87.4–99.7 %). Patients with local tumour progression/relapse after EBRT failed salvage: 5year-OS was 81.8 % (95 %CI:58.5–92.8 %) for EBRT alone and 85.3 % (95 %CI:71.6–92.7 %) for surgery and radiotherapy. Postponing LT until after chemotherapy cycle 7 did not significantly impact 5year-PFS or OS. Conclusions The risk of events was similar for different LT modalities; poor salvage after EBRT significantly reduced 5year-OS. Although not feasible for all, BT+ /-OSS offers an excellent prospect of cure, the best chance of organ retention while avoiding EBRT, and may be delayed for chemotherapy responsive tumours. Alice’s Arc, a children’s cancer charity focusing on rhabdomyosarcoma, United Kingdom (alicesarc.org), has funded data management and statistical analyses. 2025-10-24T10:17:42Z 2025-10-24T10:17:42Z 2025-10-24T10:17:42Z 2025-09-18T11:44:43Z 2025-09-18T11:44:43Z 2025-12 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion http://hdl.handle.net/11351/13681 EJC Paediatric Oncology;6 https://doi.org/10.1016/j.ejcped.2025.100313 Attribution-NonCommercial-NoDerivatives 4.0 International http://creativecommons.org/licenses/by-nc-nd/4.0/ info:eu-repo/semantics/openAccess Elsevier Scientia