Treatment of Pediatric, Adolescent, and Young Adult Patients With Fusion-Positive Alveolar Rhabdomyosarcoma Infiltrating Regional Lymph Nodes in the European CWS-2002P and RMS 2005 Studies and the Soft Tissue Sarcoma Registry
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| Titel: | Treatment of Pediatric, Adolescent, and Young Adult Patients With Fusion-Positive Alveolar Rhabdomyosarcoma Infiltrating Regional Lymph Nodes in the European CWS-2002P and RMS 2005 Studies and the Soft Tissue Sarcoma Registry |
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| Autoren: | Heinz, Amadeus Tilman, Ciuffolotti, Melissa, Merks, Johannes, Schönstein, Anton, Fuchs, Jörg, Minard-Colin, Veronique, Guillén Burrieza, Gabriela |
| Weitere Verfasser: | Institut Català de la Salut, Heinz AT Department of Pediatric Hematology and Oncology, University Children´s Hospital Tuebingen, Tuebingen, Germany. Stuttgart Cancer Center, Zentrum für Kinder-, Jugend- und Frauenmedizin (Olgahospital), Pädiatrie 5 (Pädiatrische Onkologie, Hämatologie, Immunologie), Klinikum der Landeshauptstadt Stuttgart, Stuttgart, Germany. Ciuffolotti M Stuttgart Cancer Center, Zentrum für Kinder-, Jugend- und Frauenmedizin (Olgahospital), Pädiatrie 2 (Pädiatrische Gastroenterologie), Klinikum der Landeshauptstadt Stuttgart, Stuttgart, Germany. Merks JHM Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands. Division of Imaging and Oncology, University Medical Center Utrecht, University of Utrecht, Utrecht, The Netherlands. Schönstein A Network Aging Research, Heidelberg University, Heidelberg, Germany. Minard-Colin V Department of Pediatric and Adolescent Oncology, Gustave Roussy, Université Paris-Saclay, Villejuif, France. Fuchs J Department of Pediatric Surgery and Urology, University Children’s Hospital, Tuebingen, Germany. Guillen G Servei de Cirurgia Pediàtrica, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain, Vall d'Hebron Barcelona Hospital Campus |
| Quelle: | Scientia |
| Verlagsinformationen: | Wiley |
| Publikationsjahr: | 2025 |
| Schlagwörter: | Registres mèdics, Registres hospitalaris, Tumors de parts toves - Tractament, Metàstasi limfàtica, Adolescents, DISEASES::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Connective and Soft Tissue::Neoplasms, Muscle Tissue::Myosarcoma::Rhabdomyosarcoma::Rhabdomyosarcoma, Alveolar, Other subheadings::Other subheadings::/therapy, ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Therapeutics::Combined Modality Therapy, AND EQUIPMENT::Investigative Techniques::Epidemiologic Methods::Data Collection::Registries, DISEASES::Neoplasms::Neoplastic Processes::Neoplasm Metastasis::Lymphatic Metastasis, CHEMICALS AND DRUGS::Amino Acids, Peptides, and Proteins::Proteins::Mutant Proteins::Mutant Chimeric Proteins::Oncogene Proteins, Fusion, NAMED GROUPS::Persons::Age Groups::Adolescent, ENFERMEDADES::neoplasias::neoplasias por tipo histológico::neoplasias de tejido conjuntivo y de tejidos blandos::neoplasias de tejido muscular::miosarcoma::rabdomiosarcoma::rabdomiosarcoma alveolar, Otros calificadores::Otros calificadores::/terapia, TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::terapéutica::tratamiento combinado, DIAGNÓSTICOS Y TERAPÉUTICOS::técnicas de investigación::métodos epidemiológicos::recopilación de datos::registros, ENFERMEDADES::neoplasias::procesos neoplásicos::metástasis neoplásica::metástasis linfática, COMPUESTOS QUÍMICOS Y DROGAS::aminoácidos, péptidos y proteínas::proteínas::proteínas mutantes::proteínas mutantes quiméricas::proteínas oncogénicas de fusión, DENOMINACIONES DE GRUPOS::personas::Grupos de Edad::adolescente |
| Beschreibung: | Pediatric patients; Alveolar rhabdomyosarcoma; Registry ; Pacients pediàtrics; Rabdomiosarcoma alveolar; Registre ; Pacientes pediátricos; Rabdomiosarcoma alveolar; Registro ; Background: Patients with alveolar rhabdomyosarcoma (ARMS) with regional lymph node involvement (N1) are defined as "very-high-risk rhabdomyosarcoma" in Europe. Different chemotherapy regimens were used in European study protocols. Methods: Patients with FOXO1 fusion-positive N1 ARMS registered in the CWS-2002P study, the EpSSG RMS 2005 study, and SoTiSaR were retrospectively investigated. Patients received systemic treatment with chemotherapy (CHT) and local treatment of primary tumor (PT) and involved lymph nodes (LN) with radiotherapy (RT) and/or surgery. Kaplan-Meier estimators and Cox regression were used to examine event-free survival (EFS) and overall survival (OS) according to prognostic factors and treatment. Results: A total of 156 patients registered in RMS 2005 (n = 99), CWS-2002P (n = 20), and SoTiSaR (n = 37) between 2003 and 2020 were eligible for this analysis. Median age at diagnosis was 10.2 years [0.1-21.9]. Treatment comprised CHT with IVADo (ifosfamide, vincristine, actinomycin-D, doxorubicin, n = 93; 60%), VAIA (vincristine, actinomycin-D, ifosfamide, adriamycin/doxorubicin, n = 53; 34%) or other regimens (n = 10; 6%); resection of the PT (n = 89; 57%), LN sampling or dissection (n = 92; 59%), and/or RT (n = 139; 89%). Maintenance treatment (MT) was added in n = 99/135 (73%) patients who achieved complete remission. Five-year EFS and OS of the cohort were 45% and 47%, respectively. Age and tumor size were independent prognostic factors for EFS. Local treatment applied to the LN with surgery, RT or both significantly improved EFS (p = 0.02) and OS (p = 0.04), with no difference between the modalities (p = 0.7). Conclusions: Patients with fusion-positive N1 ARMS carry a poor prognosis. Adequate local treatment of LN improved survival. Keywords: alveolar rhabdomyosarcoma; chemotherapy; fusion‐positive rhabdomyosarcoma; ... |
| Publikationsart: | article in journal/newspaper |
| Dateibeschreibung: | application/pdf |
| Sprache: | English |
| Relation: | Pediatric Blood & Cancer;72(3); https://doi.org/10.1002/pbc.31476; https://hdl.handle.net/11351/12687; 001373821100001 |
| DOI: | 10.1002/pbc.31476 |
| Verfügbarkeit: | https://hdl.handle.net/11351/12687 https://doi.org/10.1002/pbc.31476 |
| Rights: | Attribution-NonCommercial-NoDerivatives 4.0 International ; http://creativecommons.org/licenses/by-nc-nd/4.0/ ; info:eu-repo/semantics/openAccess |
| Dokumentencode: | edsbas.75E97CCB |
| Datenbank: | BASE |
Nájsť tento článok vo Web of Science | Abstract: | Pediatric patients; Alveolar rhabdomyosarcoma; Registry ; Pacients pediàtrics; Rabdomiosarcoma alveolar; Registre ; Pacientes pediátricos; Rabdomiosarcoma alveolar; Registro ; Background: Patients with alveolar rhabdomyosarcoma (ARMS) with regional lymph node involvement (N1) are defined as "very-high-risk rhabdomyosarcoma" in Europe. Different chemotherapy regimens were used in European study protocols. Methods: Patients with FOXO1 fusion-positive N1 ARMS registered in the CWS-2002P study, the EpSSG RMS 2005 study, and SoTiSaR were retrospectively investigated. Patients received systemic treatment with chemotherapy (CHT) and local treatment of primary tumor (PT) and involved lymph nodes (LN) with radiotherapy (RT) and/or surgery. Kaplan-Meier estimators and Cox regression were used to examine event-free survival (EFS) and overall survival (OS) according to prognostic factors and treatment. Results: A total of 156 patients registered in RMS 2005 (n = 99), CWS-2002P (n = 20), and SoTiSaR (n = 37) between 2003 and 2020 were eligible for this analysis. Median age at diagnosis was 10.2 years [0.1-21.9]. Treatment comprised CHT with IVADo (ifosfamide, vincristine, actinomycin-D, doxorubicin, n = 93; 60%), VAIA (vincristine, actinomycin-D, ifosfamide, adriamycin/doxorubicin, n = 53; 34%) or other regimens (n = 10; 6%); resection of the PT (n = 89; 57%), LN sampling or dissection (n = 92; 59%), and/or RT (n = 139; 89%). Maintenance treatment (MT) was added in n = 99/135 (73%) patients who achieved complete remission. Five-year EFS and OS of the cohort were 45% and 47%, respectively. Age and tumor size were independent prognostic factors for EFS. Local treatment applied to the LN with surgery, RT or both significantly improved EFS (p = 0.02) and OS (p = 0.04), with no difference between the modalities (p = 0.7). Conclusions: Patients with fusion-positive N1 ARMS carry a poor prognosis. Adequate local treatment of LN improved survival. Keywords: alveolar rhabdomyosarcoma; chemotherapy; fusion‐positive rhabdomyosarcoma; ... |
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| DOI: | 10.1002/pbc.31476 |