Localized incompletely resected standard risk rhabdomyosarcoma in children and adolescents: Results from the European Paediatric Soft Tissue Sarcoma Study Group RMS 2005 trial-Reference-Cited by-同舟云学术

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

Published:2024-07-26 Issue: Volume: Page:
ISSN:0008-543X
Container-title:Cancer
language:en
Short-container-title:Cancer

Author:

Mandeville Henry C.¹^ORCID,Bisogno Gianni²^ORCID,Minard‐Colin Veronique³^ORCID,Alaggio Rita⁴,Ben‐Arush Myriam⁵,Chargari Cyrus⁶,Coppadoro Beatrice²,Craigie Ross⁷,Devalck Christine⁸,Ferman Sima⁹,Ferrari Andrea¹⁰^ORCID,Glosli Heidi¹¹,Alvaro Raquel Hladun¹²,Hol Marinka¹³^ORCID,Mudry Peter¹⁴,Orbach Daniel¹⁵^ORCID,Albiac Monica Ramos¹⁶,Merks Johannes H. M.¹³,Jenney Meriel E. M.¹⁷

Affiliation:

1. The Royal Marsden Hospital and The Institute of Cancer Research Surrey UK

2. Hematology Oncology Division Department of Women’s and Children’s Health University of Padova Padova Italy

3. Department of Pediatric and Adolescent Oncology INSERM U1015 Gustave Roussy Université Paris‐Saclay Villejuif France

4. Pathology Unit Department of Laboratories Bambino Gesu Children's Hospital IRCCS Rome Italy

5. Ruth Rappaport Children's Hospital Rambam Medical Center Joan and Sanford Weill Pediatric Hematology Oncology and Bone Marrow Transplantation Division Haifa Israel

6. Radiation Oncology Gustave Roussy Cancer Campus Villejuif France

7. Department of Paediatric Surgery Royal Manchester Children's Hospital Manchester UK

8. Hôpital Universitaire des Enfants Reine Fabiola ULB Brussels Belgium

9. Pediatric Oncology Department National Cancer Institute Rio de Janeiro Brazil

10. Paediatric Oncology Unit Fondazione IRCCS Istituto Nazionale Tumori Milano Italy

11. Centre for Rare Disorders Division of Paediatric and Adolescent Medicine Oslo University Hospital Oslo Norway

12. Department of Pediatric Oncology and Haematology Hospital Universitari Vall d’Hebron Barcelona Spain

13. Princess Máxima Center for Pediatric Oncology Utrecht the Netherlands

14. Pediatric Oncology Department University Hospital Brno and Faculty of Medicine Masaryk University Brno Czech Republic

15. SIREDO Oncology Center Institut Curie PSL University Paris France

16. Department of Radiotherapy Hospital Universitari Vall d’Hebron Barcelona Spain

17. Children’s Hospital for Wales Heath Park Cardiff UK

Abstract

AbstractBackgroundThe 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 MethodsLocalized 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).ResultsFrom 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.ConclusionsRadiotherapy 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.

Publisher

Wiley

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