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Alveolar rhabdomyosarcoma with regional nodal involvement: results of a combined analysis from two cooperative groups
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  • Soledad Gallego,
  • Yueh-Yun Chi,
  • Gian Luca De Salvo,
  • Minjie Li,
  • Johannes Merks,
  • David A. Rodeberg,
  • Sheila Terwisscha van Scheltinga,
  • Leo Mascarenhas,
  • Daniel Orbach,
  • Meriel Jenney,
  • Lynn Million,
  • Veronique Minard-Colin,
  • Suzanne Wolden,
  • Ilaria Zanetti,
  • David Parham,
  • Henry Mandeville,
  • Rajkumar Venkatramani,
  • Gianni Bisogno,
  • Douglas Hawkins
Soledad Gallego
University Hospital Vall d'Hebron
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Yueh-Yun Chi
Children’s Hospital Los Angeles
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Gian Luca De Salvo
Istituto Oncologico Veneto
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Minjie Li
University of Florida
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Johannes Merks
Utrecht University
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David A. Rodeberg
East Carolina University
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Sheila Terwisscha van Scheltinga
Princess Maxima Center
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Leo Mascarenhas
Children's Hospital Los Angeles
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Daniel Orbach
Institut Curie
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Meriel Jenney
University of Wales Cardiff
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Lynn Million
Stanford Hospital
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Veronique Minard-Colin
Gustave Roussy
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Suzanne Wolden
Memorial Sloan Kettering Cancer Center
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Ilaria Zanetti
University-Hospital of Padova, Division of Hematology/Oncology,
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David Parham
Children’s Hospital Los Angeles
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Henry Mandeville
The Royal Marsden NHS Foundation Trust
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Rajkumar Venkatramani
Baylor College of Medicine
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Gianni Bisogno
Division of Hematology/Oncology
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Douglas Hawkins
Seattle Children's Hospital
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Background: Treatment of children and adolescents with alveolar rhabdomyosarcoma (ARMS) and regional nodal involvement (N1) have been approached differently by North American and European cooperative groups. In order to define the better therapeutic strategy, we analyzed two studies conducted between 2005 and 2016 by the European paediatric Soft tissue sarcoma Study Group (EpSSG) and Children’s Oncology Group (COG). Methods: We retrospectively identified patients with ARMS N1 enrolled in either EpSSG RMS2005 or in COG ARST0531. Chemotherapy in RMS2005 comprised IVADo (ifosfamide, vincristine, dactinomycin, doxorubicin), IVA and maintenance (vinorelbine, cyclophosphamide); in ARST0531 it consisted on either VAC (vincristine, dactinomycin, cyclophosphamide) or VAC alternating with VI (vincristine, irinotecan). Local treatment was similar in both protocols. Results: The analysis of the clinical characteristics of 239 patients showed some differences between study groups: in RMS2005, advanced IRS Group and large tumors predominated. There were no differences in outcomes between the two groups: 5-year event-free survival (EFS), 49% (95%CI=39-59) and 44% (95%CI=30-58), and overall survival (OS), 51% (95%CI=41-61) and 53.6% (95%CI=40-68), in RMS2005 and ARST0531, respectively. In RMS2005, EFS of patients with FOXO1-positive tumors was significantly inferior to those FOXO1-negative (49.3% vs 73%, p=0.034). In contrast, in ARST0531, EFS of patients with FOXO1-positive tumors was 45% compared with 43.8% for those FOXO1-negative. Conclusions: The outcome of patients with ARMS N1 was similar using different schemas of chemotherapy. However, patients with FOXO1 fusion-negative tumors enrolled in RMS2005 showed a significantly better outcome, suggesting that this subgroup may benefit from the EpSSG strategy which included maintenance chemotherapy.

Peer review status:Published

28 Sep 2020Submitted to Pediatric Blood & Cancer
28 Sep 2020Submission Checks Completed
28 Sep 2020Assigned to Editor
01 Oct 2020Reviewer(s) Assigned
13 Oct 2020Review(s) Completed, Editorial Evaluation Pending
15 Oct 2020Editorial Decision: Revise Minor
26 Oct 2020Submission Checks Completed
26 Oct 2020Assigned to Editor
26 Oct 20201st Revision Received
28 Oct 2020Reviewer(s) Assigned
29 Oct 2020Review(s) Completed, Editorial Evaluation Pending
08 Nov 2020Editorial Decision: Accept
27 Nov 2020Published in Pediatric Blood & Cancer. 10.1002/pbc.28832