European standard clinical practice recommendations for children and adolescents with Rhabdomyosarcoma a joint EpSSG, CWS and ERN PaedCan project
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| Titel: | European standard clinical practice recommendations for children and adolescents with Rhabdomyosarcoma a joint EpSSG, CWS and ERN PaedCan project |
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| Autoren: | Merks, Johannes, Brack, Eva Katharina, Ebinger, Martin, DEFACHELLES, Anne-Sophie, Hladun Alvaro, Raquel, Minard-Colin, Veronique, Guillén Burrieza, Gabriela |
| Weitere Verfasser: | Institut Català de la Salut, Merks JHM Princess Máxima Centre for Pediatric Oncology, Utrecht, the Netherlands. Division of Imaging and Oncology, University Medical Centre Utrecht, University of Utrecht, Utrecht, the Netherlands. Brack E Pediatric Hematology/Oncology, Department of Pediatrics, Inselspital, Bern University Hospital, University of Bern, Switzerland. Ebinger M Department of Pediatric Oncology and Hematology, University Children's Hospital, Eberhard Karls University, Tuebingen, Germany. Minard-Colin V Department of Pediatric and Adolescent Oncology, Gustave Roussy, Université Paris-Saclay, Villejuif, France. Defachelles AS Department of Pediatric Oncology, Oscar Lambret Center, Lille, France. Hladun R, Guillén Burrieza G Servei d'Hematologia i Oncologia Pediàtriques, Vall d′Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain, Vall d'Hebron Barcelona Hospital Campus |
| Quelle: | Scientia |
| Verlagsinformationen: | Elsevier |
| Publikationsjahr: | 2025 |
| Schlagwörter: | Tumors de parts toves - Tractament, Infants, Adolescents, NAMED GROUPS::Persons::Age Groups::Child, NAMED GROUPS::Persons::Age Groups::Adolescent, DISEASES::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Connective and Soft Tissue::Neoplasms, Muscle Tissue::Myosarcoma::Rhabdomyosarcoma, Other subheadings::Other subheadings::/therapy, DENOMINACIONES DE GRUPOS::personas::Grupos de Edad::niño, DENOMINACIONES DE GRUPOS::personas::Grupos de Edad::adolescente, 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 |
| Beschreibung: | Rhabdomyosarcoma; Treatment recommendations; Pediatric oncology ; Rabdomiosarcoma; Recomendaciones de tratamiento; Oncología pediátrica ; Rabdomiosarcoma; Recomanacions de tractament; Oncologia pediàtrica ; Rhabdomyosarcoma (RMS) is a heterogeneous group of malignancies with specific histopathological characteristics. Distinct molecular findings help to classify RMS into PAX::FOXO1 fusion gene positive and fusion gene negative subtypes. Further new molecular subtypes give insight into the heterogeneity of these rare tumours. Multiple international clinical trials have been conducted to improve the prognosis for paediatric, adolescent, and young adult patients with RMS. The overall cure rate is around 70 % but varies dramatically between the low risk localised and very high-risk metastatic patients. New treatment approaches are needed in the High-Risk and Very High-Risk RMS disease to improve patients’ outcome. State of the art diagnostics and staging is crucial and the multimodal treatment approach in specialized paediatric-oncology centres is highly recommended. Treatment includes chemotherapy with vincristine, actinomycin, and ifosfamide with or without anthracyclines. Local treatment with the aim of microscopically complete resection and/or radiotherapy is warranted depending on patient and tumour characteristics. Maintenance treatment is recommended to (Very) High Risk groups. This consensus summarizes the standard of care diagnostic work up, multimodal treatment and surveillance recommendations for paediatric, adolescent, and young adult patients with RMS. The guideline was developed as a joint project by the European paediatric Soft tissue sarcoma Study Group (EpSSG) and the Cooperative Weichteilsarkom Studiengruppe (CWS) summarized as the European RMS working group supported by European Reference Network on Paediatric Cancer (ERN PaedCan). |
| Publikationsart: | article in journal/newspaper |
| Dateibeschreibung: | application/pdf |
| Sprache: | English |
| Relation: | EJC Paediatric Oncology;5; https://doi.org/10.1016/j.ejcped.2025.100229; https://hdl.handle.net/11351/13284 |
| DOI: | 10.1016/j.ejcped.2025.100229 |
| Verfügbarkeit: | https://hdl.handle.net/11351/13284 https://doi.org/10.1016/j.ejcped.2025.100229 |
| Rights: | Attribution-NonCommercial-NoDerivatives 4.0 International ; http://creativecommons.org/licenses/by-nc-nd/4.0/ ; info:eu-repo/semantics/openAccess |
| Dokumentencode: | edsbas.5355E2C8 |
| Datenbank: | BASE |
Nájsť tento článok vo Web of Science | Abstract: | Rhabdomyosarcoma; Treatment recommendations; Pediatric oncology ; Rabdomiosarcoma; Recomendaciones de tratamiento; Oncología pediátrica ; Rabdomiosarcoma; Recomanacions de tractament; Oncologia pediàtrica ; Rhabdomyosarcoma (RMS) is a heterogeneous group of malignancies with specific histopathological characteristics. Distinct molecular findings help to classify RMS into PAX::FOXO1 fusion gene positive and fusion gene negative subtypes. Further new molecular subtypes give insight into the heterogeneity of these rare tumours. Multiple international clinical trials have been conducted to improve the prognosis for paediatric, adolescent, and young adult patients with RMS. The overall cure rate is around 70 % but varies dramatically between the low risk localised and very high-risk metastatic patients. New treatment approaches are needed in the High-Risk and Very High-Risk RMS disease to improve patients’ outcome. State of the art diagnostics and staging is crucial and the multimodal treatment approach in specialized paediatric-oncology centres is highly recommended. Treatment includes chemotherapy with vincristine, actinomycin, and ifosfamide with or without anthracyclines. Local treatment with the aim of microscopically complete resection and/or radiotherapy is warranted depending on patient and tumour characteristics. Maintenance treatment is recommended to (Very) High Risk groups. This consensus summarizes the standard of care diagnostic work up, multimodal treatment and surveillance recommendations for paediatric, adolescent, and young adult patients with RMS. The guideline was developed as a joint project by the European paediatric Soft tissue sarcoma Study Group (EpSSG) and the Cooperative Weichteilsarkom Studiengruppe (CWS) summarized as the European RMS working group supported by European Reference Network on Paediatric Cancer (ERN PaedCan). |
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| DOI: | 10.1016/j.ejcped.2025.100229 |