Alveolar rhabdomyosarcoma with regional nodal involvement: Results of a combined analysis from two cooperative groups.
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ICR Authors
Authors
Gallego, S
Chi, Y-Y
De Salvo, GL
Li, M
Merks, JHM
Rodeberg, DA
van Scheltinga, ST
Mascarenhas, L
Orbach, D
Jenney, M
Million, L
Minard-Colin, V
Wolden, S
Zanetti, I
Parham, DM
Mandeville, H
Venkatramani, R
Bisogno, G
Hawkins, DS
European paediatric Soft tissue sarcoma Study Group and Children's Oncology Group,
Chi, Y-Y
De Salvo, GL
Li, M
Merks, JHM
Rodeberg, DA
van Scheltinga, ST
Mascarenhas, L
Orbach, D
Jenney, M
Million, L
Minard-Colin, V
Wolden, S
Zanetti, I
Parham, DM
Mandeville, H
Venkatramani, R
Bisogno, G
Hawkins, DS
European paediatric Soft tissue sarcoma Study Group and Children's Oncology Group,
Document Type
Journal Article
Date
2021-03-01
Date Accepted
2020-11-08
Abstract
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 a 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 ifosfamide + vincristine + dactinomycin + doxorubicin (IVADo), IVA and maintenance (vinorelbine, cyclophosphamide); in ARST0531, it consisted of either vincristine + dactinomycin + cyclophosphamide (VAC) or VAC alternating with vincristine + irinotecan (VI). 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 Intergroup Rhabdomyosarcoma Study Group (IRS) and large tumors predominated. There were no differences in outcomes between the two groups: 5-year event-free survival (EFS), 49% (95% confidence interval [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 with FOXO1-negative (49.3% vs 73%, P = .034). In contrast, in ARST0531, EFS of patients with FOXO1-positive tumors was 45% compared with 43.8% for those with FOXO1-negative. CONCLUSIONS: The outcome of patients with ARMS N1 was similar in both protocols. However, patients with FOXO1 fusion-negative tumors enrolled in RMS2005 showed a significantly better outcome, suggesting that different strategies of chemotherapy may have an impact in the outcome of this subgroup of patients.
Citation
Pediatric Blood and Cancer, 2021, 68 (3), pp. e28832 -
Source Title
Pediatric Blood and Cancer
Publisher
WILEY
ISSN
1545-5009
eISSN
1545-5017
1545-5017
1545-5017
Collections
Research Team
Paed & Adolesc Radiother