{"doi":"10.1002/pbc.28832","title":"Alveolar rhabdomyosarcoma with regional nodal involvement: Results of a combined analysis from two cooperative groups","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.","journal":"Pediatric Blood & Cancer","year":2020,"id":100354,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":29,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9419,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2020-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":277180,"name":"Yueh‐Yun Chi","orcid":"0000-0002-9197-5684","position":1,"is_corresponding":false},{"id":492629,"name":"Gian Luca De Salvo","orcid":"0000-0001-7451-8476","position":2,"is_corresponding":false},{"id":334997,"name":"Minjie Li","orcid":"0000-0003-0616-4826","position":3,"is_corresponding":false},{"id":492630,"name":"Johannes H. M. Merks","orcid":"0000-0001-7659-1028","position":4,"is_corresponding":false},{"id":335002,"name":"David A. Rodeberg","orcid":"0000-0003-3594-7547","position":5,"is_corresponding":false},{"id":492631,"name":"Sheila Terwisscha van Scheltinga","orcid":"0000-0001-7993-0208","position":6,"is_corresponding":false},{"id":334015,"name":"Leo Mascarenhas","orcid":"0000-0001-7790-0777","position":7,"is_corresponding":false},{"id":492632,"name":"Daniel Orbach","orcid":"0000-0002-2520-139X","position":8,"is_corresponding":false},{"id":492633,"name":"Meriel Jenney","orcid":"0000-0002-2612-4621","position":9,"is_corresponding":false},{"id":492634,"name":"Lynn Million","orcid":"0000-0002-5931-4915","position":10,"is_corresponding":false},{"id":492635,"name":"Véronique Minard‐Colin","orcid":"0000-0002-0296-5207","position":11,"is_corresponding":false},{"id":281408,"name":"Suzanne L. Wolden","orcid":"0000-0002-5234-4134","position":12,"is_corresponding":false},{"id":493295,"name":"Ilaria Zanetti","orcid":null,"position":13,"is_corresponding":false},{"id":356813,"name":"David M. Parham","orcid":"0000-0002-6912-9748","position":14,"is_corresponding":false},{"id":492636,"name":"Henry Mandeville","orcid":"0000-0001-6820-8578","position":15,"is_corresponding":false},{"id":335001,"name":"Rajkumar Venkatramani","orcid":"0000-0002-4785-106X","position":16,"is_corresponding":false},{"id":492637,"name":"Gianni Bisogno","orcid":"0000-0003-4462-5523","position":17,"is_corresponding":false},{"id":277185,"name":"Douglas S. Hawkins","orcid":"0000-0003-3602-1375","position":18,"is_corresponding":false},{"id":493296,"name":"European paediatric Soft tissue sarcoma Study Group and Children's Oncology Group","orcid":null,"position":19,"is_corresponding":false},{"id":492628,"name":"Soledad Gallego","orcid":"0000-0002-4712-9624","position":0,"is_corresponding":true}],"reference_count":31,"raw_metadata":null,"created_at":"2026-07-18T22:38:35.493878Z","pmid":"33245207","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}