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EWS-FLI1 and EWS-ERG Gene Fusions Are Associated With Similar Clinical Phenotypes in Ewing's Sarcoma

From the The Children's Hospital of Philadelphia, Philadelphia; University of Pennsylvania School of Medicine, Philadelphia, PA; Clinica Universitaria de Navarra, Pamplona, Spain; Memorial Sloan-Kettering Cancer Center, New York; Columbia University College of Physicians and Surgeons, New York, NY; Children's Cancer Research Institute, St. Anna Children's Hospital, Vienna, Austria; Department of Pediatric Hematology and Oncology, and Gerhard Domagk Institute of Pathology, Westfaehlische Wilhelms University, Muenster, Germany; University of Virginia, Charlottesville, VA; Mount Sinai Hospital, Toronto, ON, Canada; and British Columbia's, Canada Children's Hospital, Vancouver, BC, Canada.

Address reprint requests to Frederic G. Barr, MD, PhD, Department of Pathology and Laboratory Medicine, University of Pennsylvania, 36th and Hamilton Walk, Philadelphia, PA 19104-6082; email barrfg{at}mail.med.upenn.edu

PURPOSE: There are a variety of solid tumors in which alternative chromosomal translocations generate related fusion products. In alveolar rhabdomyosarcoma and synovial sarcoma, these variant fusions have been found to have major clinical significance. We investigated whether the two alternative gene fusion products, EWS-FLI1 and EWS-ERG, define different clinical subsets within the Ewing's sarcoma family of tumors.

PATIENTS AND METHODS: We selected 30 cases of Ewing's sarcoma with the EWS-ERG gene fusion and 106 cases with the EWS-FLI1 fusion. Clinical data were obtained for each case and compared with the molecular diagnostic findings.

RESULTS: There were no significant clinical differences observed between the two groups in age of diagnosis, sex, metastasis at diagnosis, primary site, event-free survival, or overall survival.

CONCLUSION: Differences in the C-terminal partner in the Ewing's sarcoma family gene fusions are not associated with significant phenotypic differences.

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