EWS-FLI1 and EWS-ERG gene fusions are associated with similar clinical phenotypes in Ewing's sarcoma

被引:120
作者
Ginsberg, JP
de Alava, E
Ladanyi, M
Wexler, LH
Kovar, H
Paulussen, M
Zoubek, A
Dockhorn-Dworniczak, B
Juergens, H
Wunder, JS
Andrulis, IL
Malik, R
Sorensen, PHB
Womer, RB
Barr, FG
机构
[1] Childrens Hosp Philadelphia, Philadelphia, PA 19104 USA
[2] Univ Penn, Dept Pathol & Lab Med, Sch Med, Philadelphia, PA 19104 USA
[3] Univ Navarra Clin, Pamplona, Spain
[4] Mem Sloan Kettering Canc Ctr, New York, NY 10021 USA
[5] Columbia Univ, Coll Phys & Surg, New York, NY USA
[6] St Anna Childrens Hosp, Childrens Canc Res Inst, A-1090 Vienna, Austria
[7] Univ Munster, Dept Pediat Hematol & Oncol, D-4400 Munster, Germany
[8] Univ Munster, Gerhard Domagk Inst Pathol, D-4400 Munster, Germany
[9] Univ Virginia, Charlottesville, VA USA
[10] Mt Sinai Hosp, Toronto, ON M5G 1X5, Canada
[11] British Columbia Childrens Hosp, Vancouver, BC V6H 3V4, Canada
关键词
D O I
10.1200/JCO.1999.17.6.1809
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
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. (C) 1999 by American Society of Clinical Oncology.
引用
收藏
页码:1809 / 1814
页数:6
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