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

• Published in: Journal of clinical oncology Vol. 17; no. 6; pp. 1809 - 1814
• Main Authors: GINSBERG, J. P, DE ALAVA, E, ANDRULIS, I. L, MALIK, R, SORENSEN, P. H. B, WOMER, R. B, BARR, F. G, LADANYI, M, WEXLER, L. H, KOVAR, H, PAULUSSEN, M, ZOUBEK, A, DOCKHORN-DWORNICZAK, B, JUERGENS, H, WUNDER, J. S
• Format: Journal Article
• Language: English
• Published: Baltimore, MD American Society of Clinical Oncology 01-06-1999; Lippincott Williams & Wilkins
• Subjects: Adolescent; Adult; Biological and medical sciences; Bone Neoplasms - diagnosis; Bone Neoplasms - genetics; Bone Neoplasms - mortality; Bone Neoplasms - therapy; Disease-Free Survival; Diseases of the osteoarticular system; DNA-Binding Proteins; Female; Humans; Male; Medical sciences; Oncogene Proteins - genetics; Oncogene Proteins, Fusion - genetics; Prognosis; Proto-Oncogene Protein c-fli-1; RNA-Binding Protein EWS; Sarcoma, Ewing - diagnosis; Sarcoma, Ewing - genetics; Sarcoma, Ewing - mortality; Sarcoma, Ewing - therapy; Survival Rate; Trans-Activators; Transcription Factors - genetics; Transcriptional Regulator ERG; Translocation, Genetic - genetics; Treatment Outcome; Tumors of striated muscle and skeleton; Human; Ewing sarcoma; Prognosis; Diseases of the osteoarticular system; Sex; Malignant tumor; Metastasis; Phenotype; Genetics; Diagnosis; Bone; Age; Hybrid gene
• ISSN: 0732-183X; 1527-7755
• DOI: 10.1200/JCO.1999.17.6.1809

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.