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

alkylator chemotherapy pediatric sarcoma radiotherapy rhabdomyosarcoma surgical resection

Journal

Cancer
ISSN: 1097-0142
Titre abrégé: Cancer
Pays: United States
ID NLM: 0374236

Informations de publication

Date de publication:
26 Jul 2024
Historique:
revised: 04 06 2024
received: 09 11 2023
accepted: 10 06 2024
medline: 26 7 2024
pubmed: 26 7 2024
entrez: 26 7 2024
Statut: aheadofprint

Résumé

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). 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 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. 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.

Sections du résumé

BACKGROUND 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 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
RESULTS 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 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.

Identifiants

pubmed: 39058728
doi: 10.1002/cncr.35497
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Subventions

Organisme : Fondazione Città della Speranza
Organisme : Alice's Arc Children's Cancer Charity

Informations de copyright

© 2024 The Author(s). Cancer published by Wiley Periodicals LLC on behalf of American Cancer Society.

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Auteurs

Henry C Mandeville (HC)

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

Gianni Bisogno (G)

Hematology Oncology Division, Department of Women's and Children's Health, University of Padova, Padova, Italy.

Veronique Minard-Colin (V)

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

Rita Alaggio (R)

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

Myriam Ben-Arush (M)

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

Cyrus Chargari (C)

Radiation Oncology, Gustave Roussy Cancer Campus, Villejuif, France.

Beatrice Coppadoro (B)

Hematology Oncology Division, Department of Women's and Children's Health, University of Padova, Padova, Italy.

Ross Craigie (R)

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

Christine Devalck (C)

Hôpital Universitaire des Enfants Reine Fabiola ULB, Brussels, Belgium.

Sima Ferman (S)

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

Andrea Ferrari (A)

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

Heidi Glosli (H)

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

Raquel Hladun Alvaro (RH)

Department of Pediatric Oncology and Haematology, Hospital Universitari Vall d'Hebron, Barcelona, Spain.

Marinka Hol (M)

Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands.

Peter Mudry (P)

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

Daniel Orbach (D)

SIREDO Oncology Center, Institut Curie, PSL University, Paris, France.

Monica Ramos Albiac (MR)

Department of Radiotherapy, Hospital Universitari Vall d'Hebron, Barcelona, Spain.

Johannes H M Merks (JHM)

Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands.

Meriel E M Jenney (MEM)

Children's Hospital for Wales, Heath Park, Cardiff, UK.

Classifications MeSH