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PMID: 10561219 Published · ppublish English Journal Article Research Support, U.S. Gov't, P.H.S.

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

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 PH, Womer RB, Barr FG

Abstract

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

MeSH Terms
Adolescent Adult Bone Neoplasms/diagnosis,genetics,mortality,therapy DNA-Binding Proteins Disease-Free Survival Female Humans Male Oncogene Proteins/genetics Oncogene Proteins, Fusion/genetics Prognosis Proto-Oncogene Protein c-fli-1 RNA-Binding Protein EWS Sarcoma, Ewing/diagnosis,genetics,mortality,therapy Survival Rate Trans-Activators Transcription Factors/genetics Transcriptional Regulator ERG Translocation, Genetic/genetics Treatment Outcome
Chemicals
DNA-Binding Proteins ERG protein, human EWS-FLI fusion protein Oncogene Proteins Oncogene Proteins, Fusion Proto-Oncogene Protein c-fli-1 RNA-Binding Protein EWS Trans-Activators Transcription Factors Transcriptional Regulator ERG
Authors & Affiliations
15 authors, click to expand affiliations / ORCID
Ginsberg J P
The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, USA.
de Alava E
Ladanyi M
Wexler L H
Kovar H
Paulussen M
Zoubek A
Dockhorn-Dworniczak B
Juergens H
Wunder J S
Andrulis I L
Malik R
Sorensen P H
Womer R B
Barr F G
Article Info
Journal
Journal of clinical oncology : official journal of the American Society of Clinical Oncology
Abbr.
J Clin Oncol
ISSN
0732-183X
Published
1999-06-00
Pages
1809-14
Language
English
Region
United States
NLM ID
8309333
Subset
IM
Grants
NCI NIH HHS · CA64202 · United States
NCI NIH HHS · CA71838 · United States
NCI NIH HHS · T32-CA09615 · United States
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