TY - JOUR
T1 - Localized incompletely resected standard risk rhabdomyosarcoma in children and adolescents
T2 - Results from the European Paediatric Soft Tissue Sarcoma Study Group RMS 2005 trial
AU - Mandeville, Henry C.
AU - Bisogno, Gianni
AU - Minard-Colin, Veronique
AU - Alaggio, Rita
AU - Ben-Arush, Myriam
AU - Chargari, Cyrus
AU - Coppadoro, Beatrice
AU - Craigie, Ross
AU - Devalck, Christine
AU - Ferman, Sima
AU - Ferrari, Andrea
AU - Glosli, Heidi
AU - Alvaro, Raquel Hladun
AU - Hol, Marinka
AU - Mudry, Peter
AU - Orbach, Daniel
AU - Albiac, Monica Ramos
AU - Merks, Johannes H.M.
AU - Jenney, Meriel E.M.
N1 - Publisher Copyright:
© 2024 The Author(s). Cancer published by Wiley Periodicals LLC on behalf of American Cancer Society.
PY - 2024/12/1
Y1 - 2024/12/1
N2 - 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.
AB - 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.
KW - alkylator chemotherapy
KW - pediatric sarcoma
KW - radiotherapy
KW - rhabdomyosarcoma
KW - surgical resection
UR - http://www.scopus.com/inward/record.url?scp=85199715833&partnerID=8YFLogxK
U2 - 10.1002/cncr.35497
DO - 10.1002/cncr.35497
M3 - Article
AN - SCOPUS:85199715833
SN - 0008-543X
VL - 130
SP - 4071
EP - 4084
JO - Cancer
JF - Cancer
IS - 23
ER -