In univariate analysis, EFS and OS were only associated with histology subtype, with 100% survival of known fusion-positive SRMS. Eleven patients (31%) relapsed and four (11%) had primary refractory disease (all ERMS). The two RT patients died of rapid disease progression. Nineteen patients (51%) had embryonal RMS (ERMS) (including three highly differentiated ERMS with PTCH deletion), eight (22%) had spindle cell RMS (SRMS) (three VGLL2-, one NTRK-, and two (B)RAF-fusions), six (16%) had alveolar RMS (ARMS) (all FOXO1- or PAX3-fusion), two had unclassified RMS, and two poorly differentiated RMS were retrospectively diagnosed as rhabdoid tumors (RT) with loss of INI1 expression. Clinical data, central pathologic review, and molecular profile including RNA sequencing were analyzed. To better understand the clinical heterogeneity of RMS in infants, an integrative clinical, histological, and molecular analysis was performed.įrom 1989 to 2015, 37 infants aged less than 6 months with a diagnosis of RMS and archival tumor materials were identified in France. Rhabdomyosarcoma (RMS) in infants is a particular entity with various clinical presentations and outcomes.
19 Department of Translational Research and Innovation, Centre Léon Bérard, Univ Lyon, Université Claude Bernard Lyon 1, INSERM 1052, CNRS 5286, Cancer Research Center of Lyon, Lyon, France. 18 Department of Pediatric and Adolescent Oncology, Oscar Lambret Centre, Lille, France. 17 Department of Pediatric and Adolescent Hematogy and Oncology, Pellegrin Hospital, Bordeaux, France. 16 Department of Pediatric and Adolescent Hematogy and Oncology, Nancy Hospital, Nancy, France. 15 Department of Pediatric and Adolescent Hematogy and Oncology, La Timone Hospital, Marseille, France. 14 Department of Pediatric and Adolescent Hematogy and Oncology, Trousseau Hospital (AP-HP), Paris, France. 13 Department of Pediatric Surgery, CHU Bicetre, AP-HP, Le Kremlin-Bicêtre, France. 12 Department of Pediatric and Adolescent Hematogy and Oncology, CHU Angers, Angers, France. 11 CRESS, UMRS1153, INSERM, Université Paris-Descartes, Paris, France. 10 French National Registry of Childhood Solid Tumors, CHU Nancy, France. 9 Department of Pediatric and Adolescent Oncology, Centre Leon Berard, Lyon, France. 8 Department of Biopathology, Trousseau Hospital, Paris, France. 7 Department of Biopatholgy, Necker Hospital, Paris, France. 6 Department of Biostatistics, Gustave Roussy (GR), Villejuif, France. 5 Institut Curie, SIREDO Oncology Center (Care, Innovation and research for children and AYA with cancer), PSL Research University, Paris, France. 4 Department of Molecular Biology, Institut Curie, Paris, France. 3 Department of Translational Research and Innovation, Centre Léon Bérard, Lyon, France. 2 Department of Biopathology and Univ Lyon, Université Claude Bernard Lyon 1, INSERM 1052, CNRS 5286, Cancer Research Center of Lyon, Lyon, France. 1 Department of Pediatric and Adolescent Oncology, Gustave Roussy (GR), Villejuif, France. Ludovic butel download#
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