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Clinical Trial
. 2024 Dec 1;130(23):4071-4084.
doi: 10.1002/cncr.35497. Epub 2024 Jul 26.

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

Henry C Mandeville  1 , Gianni Bisogno  2 , Veronique Minard-Colin  3 , Rita Alaggio  4 , Myriam Ben-Arush  5 , Cyrus Chargari  6 , Beatrice Coppadoro  2 , Ross Craigie  7 , Christine Devalck  8 , Sima Ferman  9 , Andrea Ferrari  10 , Heidi Glosli  11 , Raquel Hladun Alvaro  12 , Marinka Hol  13 , Peter Mudry  14 , Daniel Orbach  15 , Monica Ramos Albiac  16 , Johannes H M Merks  13 , Meriel E M Jenney  17
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Free article
Clinical Trial

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

Henry C Mandeville et al. Cancer. .
Free article

Abstract

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.

Keywords: alkylator chemotherapy; pediatric sarcoma; radiotherapy; rhabdomyosarcoma; surgical resection.

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References

REFERENCES

    1. Pastore G, Peris‐Bonet R, Carli M, Martínez‐García C, de Toledo JS, Steliarova‐Foucher E. Childhood soft tissue sarcomas incidence and survival in European children (1978‐1997): report from the Automated Childhood Cancer Information System project. Eur J Cancer. 2006;42(13):2136‐2149. doi:10.1016/j.ejca.2006.05.016
    1. Stiller CA, Stevens MCG, Magnani C, Corazziari I. Survival of children with soft‐tissue sarcoma in Europe since 1978: results from the EUROCARE study. Eur J Cancer. 2001;37(6):767‐774. doi:10.1016/S0959‐8049(01)00007‐7
    1. Skapek SX, Ferrari A, Gupta AA, et al. Rhabdomyosarcoma. Nat Rev Dis Primers. 2019;5(1):1. doi:10.1038/s41572‐018‐0051‐2
    1. Cecchetto G, Bisogno G, De Corti F, et al. Biopsy or debulking surgery as initial surgery for locally advanced rhabdomyosarcomas in children? Cancer. 2007;110(11):2561‐2567. doi:10.1002/cncr.23079
    1. Cecchetto G, Bisogno G, Treuner J, et al. Role of surgery for nonmetastatic abdominal rhabdomyosarcomas. Cancer. 2003;97(8):1974‐1980. doi:10.1002/cncr.11285

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