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. 2022 Nov;69(11):e29853.
doi: 10.1002/pbc.29853. Epub 2022 Jun 30.

Metastatic rhabdomyosarcoma: Evidence of the impact of radiotherapy on survival. A retrospective single-center experience

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Metastatic rhabdomyosarcoma: Evidence of the impact of radiotherapy on survival. A retrospective single-center experience

Andrea Ferrari et al. Pediatr Blood Cancer. 2022 Nov.

Abstract

Background: The prognosis for patients with metastatic rhabdomyosarcoma (RMS) remains largely unsatisfactory despite the adoption of intensive multimodal therapy. To assess the role of different treatments adopted over the years, we retrospectively analyzed a cohort of patients <21 years old with metastatic RMS, treated from 1990 to 2020 at a referral center for pediatric sarcomas.

Methods: Patients were treated using a multimodal approach that included surgery, radiotherapy, and chemotherapy (both high-dose chemotherapy and maintenance therapy in some cases). The type of radiotherapy administered was categorized as radical (to all sites of disease); partial (to at least one, but not all sites of disease); or none. A landmark analysis was used to examine the impact of radiotherapy on survival, that is, patients who had an event before day 221 were excluded from the analysis.

Results: The series included 80 patients. Event-free survival (EFS) and overall survival (OS) rates at 5 years were 17.3% and 21.3%, respectively. Survival was significantly associated with radiotherapy to metastatic sites, and with the radiotherapy category. In particular, 5-year EFS and OS rates were 70.6% and 76.0% for patients given radical radiotherapy, and 4.8% and 10.7%, respectively, for those given partial radiotherapy or none. Using the Cox multivariable analysis, OS correlated significantly with radiotherapy category.

Conclusions: While confirming the poor overall outcome of patients with metastatic RMS, this study identified radiotherapy-when given to all sites of disease (including metastases)-as the main variable influencing survival.

Keywords: maintenance chemotherapy; metastases; metastatic rhabdomyosarcoma; outcome; prognostic factors; radiotherapy; rhabdomyosarcoma; treatment.

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References

REFERENCES

    1. Skapek S, Ferrari A, Gupta A, et al. Rhabdomyosarcoma. Nat Rev Dis Primers. 2019;5(1):1. https://doi.org/10.1038/s41572-018-0051-2
    1. Ferrari A, Sultan I, Huang TT, et al. Soft tissue sarcoma across the age spectrum: a population-based study from the Surveillance Epidemiology and End Results database. Pediatr Blood Cancer. 2011;57:943-949.
    1. Ferrari A, Brecht IB, Gatta G, et al. Defining and listing very rare cancers of pediatric age: consensus of the Joint Action on Rare Cancers (JARC) in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT). Eur J Cancer. 2019;110:120-126.
    1. Ferrari A, Trama A, De Paoli A, et al. Access to clinical trials for adolescents with soft tissue sarcomas: enrolment in European pediatric Soft tissue sarcoma Study Group (EpSSG) protocols. Pediatr Blood Cancer. 2017;64:6.
    1. Ferrari A, Dama E, Pession A, et al. Adolescents with cancer in Italy: entry into the national cooperative pediatric oncology group AIEOP trials. Eur J Cancer. 2009;45:328-334.

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