Publication details

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

Authors	MANDEVILLE Henry C BISOGNO Gianni MINARD-COLIN Veronique ALAGGIO Rita BEN-ARUSH Myriam CHARGARI Cyrus COPPADORO Beatrice CRAIGIE Ross DEVALCK Christine FERMAN Sima FERRARI Andrea GLOSLI Heidi ALVARO Raquel Hladun HOL Marinka MÚDRY Peter ORBACH Daniel ALBIAC Monica Ramos MERKS Johannes H M JENNEY Meriel E M
Year of publication	2024
Type	Article in Periodical
Magazine / Source	Cancer
MU Faculty or unit	Faculty of Medicine
Citation
Web	https://acsjournals.onlinelibrary.wiley.com/doi/10.1002/cncr.35497
Doi	http://dx.doi.org/10.1002/cncr.35497
Keywords	alkylator chemotherapy; pediatric sarcoma; radiotherapy; rhabdomyosarcoma; surgical resection
Description	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/m(2) ifosfamide) and four cycles of vincristine and dactinomycin (if receiving radiotherapy), or nine cycles of IVA (54 g/m(2) 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.