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


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.


CONCLUSIONS
Differences in the C-terminal partner in the Ewing's sarcoma family gene fusions are not associated with significant phenotypic differences.
Ginsberg
J P
JP
The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, USA.
de Alava
E
E
Ladanyi
M
M
Wexler
L H
LH
Kovar
H
H
Paulussen
M
M
Zoubek
A
A
Dockhorn-Dworniczak
B
B
Juergens
H
H
Wunder
J S
JS
Andrulis
I L
IL
Malik
R
R
Sorensen
P H
PH
Womer
R B
RB
Barr
F G
FG
D000293:Adolescent; D000328:Adult; D001859:Bone Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000401:mortality / Q000628:therapy; D004268:DNA-Binding Proteins; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D015513:Oncogene Proteins / Q000235:genetics*; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D011379:Prognosis; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D012512:Sarcoma, Ewing / Q000175:diagnosis / Q000235:genetics* / Q000401:mortality / Q000628:therapy; D015996:Survival Rate; D015534:Trans-Activators; D014157:Transcription Factors / Q000235:genetics*; D000071230:Transcriptional Regulator ERG; D014178:Translocation, Genetic / Q000235:genetics; D016896:Treatment Outcome
D004268:DNA-Binding Proteins; C072312:ERG protein, human; C106031:EWS-FLI fusion protein; D015513:Oncogene Proteins; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D015534:Trans-Activators; D014157:Transcription Factors; D000071230:Transcriptional Regulator ERG