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.

Autor: 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; Pediatric Surgery Department, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain., Timmermann B; Department of Particle Therapy, West German Proton Therapy Centre Essen (WPE), University Medical Center Essen, West German Cancer Center (WTZ), German Cancer Consortium (DKTK), Essen, Germany., Vokuhl C; Section of Pediatric Pathology, Department of Pathology, University Bonn, Bonn, Germany., Koscielniak E; Stuttgart Cancer Center, Zentrum für Kinder-, Jugend- und Frauenmedizin (Olgahospital), Pädiatrie 5 (Pädiatrische Onkologie, Hämatologie, Immunologie), Klinikum der Landeshauptstadt Stuttgart, Stuttgart, Germany.; Medical Faculty, University Tuebingen, Tuebingen, Germany., Chisholm JC; Children and Young People's Unit, The Royal Marsden NHS Foundation Trust and Institute of Cancer Research, Sutton, UK., Sparber-Sauer M; Stuttgart Cancer Center, Zentrum für Kinder-, Jugend- und Frauenmedizin (Olgahospital), Pädiatrie 5 (Pädiatrische Onkologie, Hämatologie, Immunologie), Klinikum der Landeshauptstadt Stuttgart, Stuttgart, Germany.; Medical Faculty, University Tuebingen, Tuebingen, Germany., 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.
Jazyk: angličtina
Zdroj: Pediatric blood & cancer [Pediatr Blood Cancer] 2024 Dec 10, pp. e31476. Date of Electronic Publication: 2024 Dec 10.
DOI: 10.1002/pbc.31476
Abstrakt: 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.
(© 2024 The Author(s). Pediatric Blood & Cancer published by Wiley Periodicals LLC.)
Databáze: MEDLINE
Externí odkaz: