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      Current treatment protocols have eliminated the prognostic advantage of type 1 fusions in Ewing sarcoma: a report from the Children's Oncology Group.

      Journal of clinical oncology : official journal of the American Society of Clinical Oncology
      Adolescent, Adult, Antineoplastic Combined Chemotherapy Protocols, therapeutic use, Biopsy, Bone Neoplasms, genetics, mortality, pathology, therapy, Chi-Square Distribution, Child, Child, Preschool, Disease-Free Survival, Female, Gene Expression Regulation, Neoplastic, Genetic Predisposition to Disease, Humans, Infant, Kaplan-Meier Estimate, Logistic Models, Male, Oncogene Proteins, Fusion, Phenotype, Prospective Studies, Proto-Oncogene Protein c-fli-1, RNA-Binding Protein EWS, Reverse Transcriptase Polymerase Chain Reaction, Risk Assessment, Risk Factors, Sarcoma, Ewing, secondary, Time Factors, Transcription Factors, Treatment Outcome, United States, Young Adult

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          Abstract

          PURPOSE Ewing sarcoma family tumors (ESFTs) exhibit chromosomal translocations that lead to the creation of chimeric fusion oncogenes. Combinatorial diversity among chromosomal breakpoints produces varying fusions. The type 1 EWS-FLI1 transcript is created as a result of fusion between exons 7 of EWS and 6 of FLI1, and retrospective studies have reported that type 1 tumors are associated with an improved outcome. We have re-examined this association in a prospective cohort of patients with ESFT treated according to current Children's Oncology Group (COG) treatment protocols. METHODS Frozen tumor tissue was prospectively obtained from patients diagnosed with ESFT, and reverse transcriptase polymerase chain reaction (RT-PCR) was used to determine translocation status. Analysis was confined to patients with localized tumors who were diagnosed after 1994 and treated according to COG protocols. Translocation status was correlated with disease characteristics, event-free survival (EFS), and overall survival (OS). Results RT-PCR identified chimeric fusion oncogenes in 119 of 132 ESFTs. Eighty-nine percent of identified transcripts were EWS-FLI1, and of these, 58.8% were type 1. Five-year EFS and OS rates for patients with type 1 and non-type 1 fusions diagnosed between 2001 and 2005 were equivalent (type 1: EFS, 63% +/- 7%; OS, 83% +/- 6%; non-type 1: EFS, 71% +/- 9%; OS, 79% +/- 8%). CONCLUSION Current intensive treatment protocols for localized ESFT have erased the clinical disadvantage that was formerly observed in patients with non-type 1 EWS-FLI1 fusions.

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