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Multicenter Study
. 2021 Mar;68(3):e28832.
doi: 10.1002/pbc.28832. Epub 2020 Nov 27.

Alveolar rhabdomyosarcoma with regional nodal involvement: Results of a combined analysis from two cooperative groups

Soledad Gallego  1 , Yueh-Yun Chi  2 , Gian Luca De Salvo  3 , Minjie Li  4 , Johannes H M Merks  5 , David A Rodeberg  6 , Sheila Terwisscha van Scheltinga  5 , Leo Mascarenhas  2 , Daniel Orbach  7 , Meriel Jenney  8 , Lynn Million  9 , Veronique Minard-Colin  10 , Suzanne Wolden  11 , Ilaria Zanetti  12 , David M Parham  2 , Henry Mandeville  13 , Rajkumar Venkatramani  14 , Gianni Bisogno  12 , Douglas S Hawkins  15 ; European paediatric Soft tissue sarcoma Study Group and Children's Oncology Group  1
Affiliations
Multicenter Study

Alveolar rhabdomyosarcoma with regional nodal involvement: Results of a combined analysis from two cooperative groups

Soledad Gallego et al. Pediatr Blood Cancer. 2021 Mar.

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.

Keywords: alveolar rhabdomyosarcoma; chemotherapy; nodal involvement; prognostic factors; rhabdomyosarcoma.

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Conflict of interest statement

Potential conflicts of interest: The author(s) indicated no potential conflicts of interest.

Figures

Figure 1
Figure 1
Kaplan-Meier curves representing (A) 5-year EFS and (B) 5-year OS, by protocol.
Figure 2
Figure 2
Kaplan-Meier curves representing 5-year EFS by fusion status in separated cohorts, (A) EpSSG RMS2005 and (B) COG ARST0531.
Figure 3
Figure 3
Kaplan-Meier curves representing 5-year OS by fusion status in separated cohorts (A) EpSSG RMS2005 and (B) COG ARST0531.
See this image and copyright information in PMC

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