PMID- 33245207 OWN - NLM STAT- MEDLINE DCOM- 20210802 LR - 20220323 IS - 1545-5017 (Electronic) IS - 1545-5009 (Print) IS - 1545-5009 (Linking) VI - 68 IP - 3 DP - 2021 Mar TI - Alveolar rhabdomyosarcoma with regional nodal involvement: Results of a combined analysis from two cooperative groups. PG - e28832 LID - 10.1002/pbc.28832 [doi] AB - 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. CI - (c) 2020 Wiley Periodicals LLC. FAU - Gallego, Soledad AU - Gallego S AUID- ORCID: 0000-0002-4712-9624 AD - University Hospital Vall d'Hebron, Barcelona, Spain. FAU - Chi, Yueh-Yun AU - Chi YY AD - Children's Hospital Los Angeles, University of Southern California, Los Angeles, California. FAU - De Salvo, Gian Luca AU - De Salvo GL AD - Clinical Trials and Biostatistics Unit, Veneto Oncologic Institute IOV-IRCCS, Padua, Italy. FAU - Li, Minjie AU - Li M AD - University of Florida, Gainesville, Florida. FAU - Merks, Johannes H M AU - Merks JHM AD - Princess Maxima Center for Pediatric Oncology, Utrecht, The Netherlands. FAU - Rodeberg, David A AU - Rodeberg DA AUID- ORCID: 0000-0003-3594-7547 AD - East Carolina University, Greenville, North Carolina. FAU - van Scheltinga, Sheila Terwisscha AU - van Scheltinga ST AD - Princess Maxima Center for Pediatric Oncology, Utrecht, The Netherlands. FAU - Mascarenhas, Leo AU - Mascarenhas L AD - Children's Hospital Los Angeles, University of Southern California, Los Angeles, California. FAU - Orbach, Daniel AU - Orbach D AD - SIREDO Oncology Center, Institut Curie, PSL University, Paris, France. FAU - Jenney, Meriel AU - Jenney M AD - Children Hospital for Wales Cardiff and Vale University Health Board, Cardiff, UK. FAU - Million, Lynn AU - Million L AD - Stanford University School of Medicine, Stanford, California. FAU - Minard-Colin, Veronique AU - Minard-Colin V AUID- ORCID: 0000-0002-0296-5207 AD - Gustave Roussy, University Paris-Saclay, Villejuif, France. FAU - Wolden, Suzanne AU - Wolden S AUID- ORCID: 0000-0002-5234-4134 AD - Memorial Sloan Kettering Cancer Center, New York, New York. FAU - Zanetti, Ilaria AU - Zanetti I AD - Padova University Hospital, Padua, Italy. FAU - Parham, David M AU - Parham DM AD - Children's Hospital Los Angeles, University of Southern California, Los Angeles, California. FAU - Mandeville, Henry AU - Mandeville H AD - The Royal Marsden NHS Foundation Trust, Sutton, UK. FAU - Venkatramani, Rajkumar AU - Venkatramani R AD - Texas Children's Hospital, Baylor College of Medicine, Houston, Texas. FAU - Bisogno, Gianni AU - Bisogno G AUID- ORCID: 0000-0003-4462-5523 AD - Padova University Hospital, Padua, Italy. FAU - Hawkins, Douglas S AU - Hawkins DS AUID- ORCID: 0000-0003-3602-1375 AD - Seattle Children's Hospital, Fred Hutchinson Cancer Research Center, University of Washington, Seattle, Washington. CN - European paediatric Soft tissue sarcoma Study Group and Children's Oncology Group AD - University Hospital Vall d'Hebron, Barcelona, Spain. LA - eng GR - U10CA098543/NH/NIH HHS/United States GR - 5943/CRUK_/Cancer Research UK/United Kingdom GR - P30 CA008748/CA/NCI NIH HHS/United States GR - U10CA098413/NH/NIH HHS/United States GR - U10 CA098543/CA/NCI NIH HHS/United States GR - U10 CA180899/CA/NCI NIH HHS/United States GR - U10 CA180886/CA/NCI NIH HHS/United States GR - U10 CA098413/CA/NCI NIH HHS/United States GR - U10CA180899/NH/NIH HHS/United States GR - U10CA180886/NH/NIH HHS/United States PT - Journal Article PT - Multicenter Study PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20201127 PL - United States TA - Pediatr Blood Cancer JT - Pediatric blood & cancer JID - 101186624 SB - IM MH - Antineoplastic Combined Chemotherapy Protocols/*therapeutic use MH - Child MH - Child, Preschool MH - Female MH - Follow-Up Studies MH - Humans MH - Infant MH - Infant, Newborn MH - Lymph Nodes/*pathology MH - Male MH - Prognosis MH - Retrospective Studies MH - Rhabdomyosarcoma, Alveolar/drug therapy/*mortality/pathology MH - Survival Rate PMC - PMC8414760 MID - NIHMS1734154 OTO - NOTNLM OT - alveolar rhabdomyosarcoma OT - chemotherapy OT - nodal involvement OT - prognostic factors OT - rhabdomyosarcoma COIS- Potential conflicts of interest: The author(s) indicated no potential conflicts of interest. EDAT- 2020/11/28 06:00 MHDA- 2021/08/03 06:00 PMCR- 2022/03/01 CRDT- 2020/11/27 08:38 PHST- 2020/09/28 00:00 [received] PHST- 2020/10/26 00:00 [revised] PHST- 2020/11/08 00:00 [accepted] PHST- 2020/11/28 06:00 [pubmed] PHST- 2021/08/03 06:00 [medline] PHST- 2020/11/27 08:38 [entrez] PHST- 2022/03/01 00:00 [pmc-release] AID - 10.1002/pbc.28832 [doi] PST - ppublish SO - Pediatr Blood Cancer. 2021 Mar;68(3):e28832. doi: 10.1002/pbc.28832. Epub 2020 Nov 27.