Sistema de Salut de Catalunya
Institut Català de la Salut
[de Vries ISA] Princess Máxima Center for Pediatric Oncology, CS Utrecht, Netherlands. [Morosi C] Department of Radiology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. [Bisogno G] Department of Women’s and Children’s Health, University of Padua, Padua, Italy. Pediatric Hematology Oncology Division, University Hospital of Padua, Padua, Italy. [Minard Colin V] Department of Pediatric and Adolescent Oncology, Gustave-Roussy, Université Paris-Saclay, Villejuif, France. [Coppadoro B] Department of Women’s and Children’s Health, University of Padua, Padua, Italy. [Zanetti I] Pediatric Hematology Oncology Division, University Hospital of Padua, Padua, Italy. [Guillen G] Servei de Cirurgia Pediàtrica, Vall d’Hebron Hospital Universitari, Barcelona, Spain
Vall d'Hebron Barcelona Hospital Campus
2025-10-31T07:25:50Z
2025-10-31T07:25:50Z
2025-09
Paediatric oncology; Radiological response assessment; Rhabdomyosarcoma
Oncología pediátrica; Evaluación de la respuesta radiológica; Rabdomiosarcoma
Oncologia pediàtrica; Avaluació de la resposta radiològica; Rabdomiosarcoma
Background There is an urgent need for early radiological markers predicting survival in paediatric rhabdomyosarcoma patients. Objective To analyse the predictive value of early radiological tumour volume response to chemotherapy in non-metastatic rhabdomyosarcoma patients. Methods The European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 study prospectively included non-metastatic rhabdomyosarcoma patients. Patients with Intergroup Rhabdomyosarcoma Studies (IRS) postsurgical Group III (i.e. measurable disease) at diagnosis, with three tumour diameters available at diagnosis and following three cycles of chemotherapy, were included. Tumour response was categorized as complete response, partial response, minor partial response, stable disease, or progressive disease. Patients with progressive disease were excluded from analyses. Failure-free survival and overall survival were estimated with Kaplan-Meier using a landmark analysis. Prognostic factors were assessed using Cox proportional hazards analyses. Results Of 1,674 patients aged < 18 years at diagnosis included in the EpSSG RMS 2005 study, 613 patients (median age 4.2 years, interquartile range 2.2–8.0, 346 males) were eligible for analysis. After three cycles of chemotherapy, 48 patients reached complete response, 371 partial response, 139 minor partial response, 48 stable disease, and seven patients progressive disease. Univariate Cox proportional hazards model showed no significant difference between response groups for failure-free survival or overall survival. In multivariate Cox regression analysis, tumour volume response was not an independent prognostic factor for failure-free survival or overall survival. Conclusion Early radiological tumour volume response after the third cycle of neoadjuvant chemotherapy, with exclusion of progressive disease, does not predict survival in paediatric rhabdomyosarcoma patients.
Alice's Arc, a children’s cancer charity focusing on rhabdomyosarcoma, United Kingdom (alicesarc.org), has funded data management and statistical processing for current analyses.
Article
Published version
English
Tumors de parts toves - Quimioteràpia; Tumors de parts toves - Radioteràpia; Tumors de parts toves - Prognosi; 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::Investigative Techniques::Anthropometry::Body Weights and Measures::Tumor Burden; ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Therapeutics::Radiotherapy; ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Diagnosis::Prognosis; 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::técnicas de investigación::antropometría::pesos y medidas corporales::carga tumoral; TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::terapéutica::radioterapia; TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::diagnóstico::pronóstico
Springer
Pediatric Radiology;55
https://doi.org/10.1007/s00247-025-06359-3
Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
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