This Article Full Text Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Ginsberg, J. P. Articles by Barr, F. G. Search for Related Content PubMed PubMed Citation Articles by Ginsberg, J. P. Articles by Barr, F. G. Social Bookmarking                            What's this?

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.

CiteULike    Complore    Connotea    Del.icio.us    Digg    Facebook    Reddit    Technorati    Twitter    What's this?

This article has been cited by other articles:

				E. A. Kruse, S. J. Loughran, T. M. Baldwin, E. C. Josefsson, S. Ellis, D. K. Watson, P. Nurden, D. Metcalf, D. J. Hilton, W. S. Alexander, et al. Dual requirement for the ETS transcription factors Fli-1 and Erg in hematopoietic stem cells and the megakaryocyte lineage PNAS, August 18, 2009; 106(33): 13814 - 13819. [Abstract] [Full Text] [PDF]

				O. Slater and J. Shipley Clinical relevance of molecular genetics to paediatric sarcomas J. Clin. Pathol., November 1, 2007; 60(11): 1187 - 1194. [Abstract] [Full Text] [PDF]

				T. L. Ng, M. J. O'Sullivan, C. J. Pallen, M. Hayes, P. W. Clarkson, M. Winstanley, P. H.B. Sorensen, T. O. Nielsen, and D. E. Horsman Ewing Sarcoma with Novel Translocation t(2;16) Producing an In-Frame Fusion of FUS and FEV J. Mol. Diagn., September 1, 2007; 9(4): 459 - 463. [Abstract] [Full Text] [PDF]

				F. G. Barr and R. B. Womer Molecular Diagnosis of Ewing Family Tumors: Too Many Fusions... ? J. Mol. Diagn., September 1, 2007; 9(4): 437 - 440. [Abstract] [Full Text] [PDF]

				L. Wang, R. Bhargava, T. Zheng, L. Wexler, M. H. Collins, D. Roulston, and M. Ladanyi Undifferentiated Small Round Cell Sarcomas with Rare EWS Gene Fusions: Identification of a Novel EWS-SP3 Fusion and of Additional Cases with the EWS-ETV1 and EWS-FEV Fusions J. Mol. Diagn., September 1, 2007; 9(4): 498 - 509. [Abstract] [Full Text] [PDF]

				Y. Iwamoto Diagnosis and Treatment of Ewing's Sarcoma Jpn. J. Clin. Oncol., February 1, 2007; 37(2): 79 - 89. [Abstract] [Full Text] [PDF]

				M. Scurr and I. Judson How to Treat the Ewing's Family of Sarcomas in Adult Patients Oncologist, January 1, 2006; 11(1): 65 - 72. [Abstract] [Full Text] [PDF]

				L. Rainis, T. Toki, J. E. Pimanda, E. Rosenthal, K. Machol, S. Strehl, B. Gottgens, E. Ito, and S. Izraeli The Proto-Oncogene ERG in Megakaryoblastic Leukemias Cancer Res., September 1, 2005; 65(17): 7596 - 7602. [Abstract] [Full Text] [PDF]

				H.-Y. Huang, P. B. Illei, Z. Zhao, M. Mazumdar, A. G. Huvos, J. H. Healey, L. H. Wexler, R. Gorlick, P. Meyers, and M. Ladanyi Ewing Sarcomas With p53 Mutation or p16/p14ARF Homozygous Deletion: A Highly Lethal Subset Associated With Poor Chemoresponse J. Clin. Oncol., January 20, 2005; 23(3): 548 - 558. [Abstract] [Full Text] [PDF]

				A. M. Oliveira and C. D.M. Fletcher Molecular Prognostication for Soft Tissue Sarcomas: Are We Ready Yet? J. Clin. Oncol., October 15, 2004; 22(20): 4031 - 4034. [Full Text] [PDF]

				Y. Matsui, H. A. Chansky, F. Barahmand-Pour, A. Zielinska-Kwiatkowska, N. Tsumaki, A. Myoui, H. Yoshikawa, L. Yang, and D. R. Eyre COL11A2 Collagen Gene Transcription Is Differentially Regulated by EWS/ERG Sarcoma Fusion Protein and Wild-type ERG J. Biol. Chem., March 21, 2003; 278(13): 11369 - 11375. [Abstract] [Full Text] [PDF]

				S A Burchill Ewing's sarcoma: diagnostic, prognostic, and therapeutic implications of molecular abnormalities J. Clin. Pathol., February 1, 2003; 56(2): 96 - 102. [Abstract] [Full Text] [PDF]

				P. H.B. Sorensen, J. C. Lynch, S. J. Qualman, R. Tirabosco, J. F. Lim, H. M. Maurer, J. A. Bridge, W. M. Crist, T. J. Triche, and F. G. Barr PAX3-FKHR and PAX7-FKHR Gene Fusions Are Prognostic Indicators in Alveolar Rhabdomyosarcoma: A Report From the Children's Oncology Group J. Clin. Oncol., June 1, 2002; 20(11): 2672 - 2679. [Abstract] [Full Text] [PDF]

				M. C. Manara, B. Perbal, S. Benini, R. Strammiello, V. Cerisano, S. Perdichizzi, M. Serra, A. Astolfi, F. Bertoni, J. Alami, et al. The Expression of ccn3(nov) Gene in Musculoskeletal Tumors Am. J. Pathol., March 1, 2002; 160(3): 849 - 859. [Abstract] [Full Text] [PDF]

				E. de Alava and J. Pardo Ewing Tumor: Tumor Biology and Clinical Applications International Journal of Surgical Pathology, January 1, 2001; 9(1): 7 - 17. [Abstract] [PDF]

				E. de Alava, A. Panizo, C. R. Antonescu, A. G. Huvos, F. J. Pardo-Mindan, F. G. Barr, and M. Ladanyi Association of EWS-FLI1 Type 1 Fusion with Lower Proliferative Rate in Ewing’s Sarcoma Am. J. Pathol., March 1, 2000; 156(3): 849 - 855. [Abstract] [Full Text] [PDF]