Localized incompletely resected standard risk rhabdomyosarcoma in children and adolescents: Results from the European Paediatric Soft Tissue Sarcoma Study Group RMS 2005 trial

Other authors

Institut Català de la Salut

[Mandeville HC] The Royal Marsden Hospital and The Institute of Cancer Research, Surrey, UK. [Bisogno G] Hematology Oncology Division, Department of Women’s and Children’s Health, University of Padova, Padova, Italy. [Minard‐Colin V] Department of Pediatric and Adolescent Oncology, INSERM U1015, Gustave Roussy, Université Paris‐Saclay, Villejuif, France. [Alaggio R] Pathology Unit, Department of Laboratories, Bambino Gesu Children's Hospital, IRCCS, Rome, Italy. [Ben‐Arush M] Ruth Rappaport Children's Hospital, Rambam Medical Center, Joan and Sanford Weill Pediatric Hematology Oncology and Bone Marrow Transplantation Division, Haifa, Israel. [Chargari C] Radiation Oncology, Gustave Roussy Cancer Campus, Villejuif, France. [Hladun Alvaro R] Servei d'Hematologia i Oncologia Pediàtriques, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Ramos Albiac M] Servei de Radiodiagnòstic, Vall d’Hebron Hospital Universitari, Barcelona, Spain

Vall d'Hebron Barcelona Hospital Campus

Publication date

2025-01-17T13:10:18Z

2025-01-17T13:10:18Z

2024-12-01



Abstract

Alkylator chemotherapy; Radiotherapy; Rhabdomyosarcoma


Quimioterapia alquilante; Radioterapia; Rabdomiosarcoma


Quimioteràpia alquilant; Radioteràpia; Rabdomiosarcoma


Background The authors report the prospective evaluation of reduced dose alkylator chemotherapy combined with radiotherapy for European Pediatric Soft Tissue Sarcoma Study Group (EpSSG) standard risk nonalveolar rhabdomyosarcoma (NA-RMS). Patients and Methods Localized node negative Intergroup Rhabdomyosarcoma Study (IRS) II/III NA-RMS at favorable sites (subgroup C), <25 years old, received five cycles of ifosfamide, vincristine, and dactinomycin (IVA) chemotherapy (30 g/m2 ifosfamide) and four cycles of vincristine and dactinomycin (if receiving radiotherapy), or nine cycles of IVA (54 g/m2 ifosfamide) ± radiotherapy. Delayed primary tumor excision was considered for IRS III tumors. The primary end points were event-free survival (EFS) and overall survival (OS). Results From October 2005 to December 2016, 359 evaluable patients were recruited: orbit, 164 (45.7%); head and neck nonparameningeal, 77 (21.4%); and genitourinary non–bladder/prostate, 118 (32.9%). EFS and OS were 77.4% (95% confidence interval [CI], 72.5–81.6) and 93.5% (95% CI, 90.1–95.8), respectively. Lower dose alkylator chemotherapy and radiotherapy achieved 5-year OS of 93.7% but the difference with higher dose alkylator chemotherapy +/- radiotherapy was not significant (p = 0.8003). Adjuvant radiotherapy improved EFS with 5-year estimates of 84.7% versus 65.2% for nonirradiated (p < .0001), but not OS (p = .9298). Omitting radiotherapy for orbital tumors reduced OS (5-year was 87.1% vs. 97.3% for irradiated, p = .0257). Following R0 resection (n = 60), radiotherapy did not significantly improve EFS or OS. Conclusions Radiotherapy for local tumor control allows for reduction of cumulative dose of alkylators in EpSSG standard risk subgroup C RMS patients. The omission of radiotherapy did not affect OS in all patients except those with orbital RMS and was associated with inferior EFS.

Document Type

Article


Published version

Language

English

Publisher

Wiley

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Attribution 4.0 International

http://creativecommons.org/licenses/by/4.0/

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