Key result
Recent advances reveal EWS-FLI1 fusion mitogenic targets, splicing deregulation, and p53 modulation in Ewing's sarcoma.
Why the study?
Recent advances in understanding the EWS-FLI1 fusion oncogene in Ewing's sarcoma have revealed new complexities in tumor biology relevant to fusion oncogenes.
This review summarizes molecular insights into the EWS-FLI1 fusion oncogene in Ewing's sarcoma, including its role in splicing and interaction with the p53 pathway.
Preclinical EWS-FLI1 insights warrant no practice change; leaves open targeted therapy development in Ewing sarcoma.
The EWS-FLI1 fusion resulting from the specific t(11;22)(q24;q12) of Ewing's sarcoma was the first sarcoma gene fusion to be cloned, a decade ago. Moving from the cloning of this oncogenic chimeric transcription factor to the further elucidation of its pathogenetic mechanisms has revealed new complexities in the biology of this tumor which may be relevant to other fusion oncogenes as well. The present review highlights recent advances in three avenues of investigation that are providing new insights into this particular neoplasm and into the biology of EWS-FLI1 and related fusion proteins, namely the identification of various mitogenic targets of this aberrant transcription factor, its possible role as a deregulator of splicing, and the role of the p53 pathway in modulating its oncogenicity.
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Marc Ladanyi (2002) conducted a review in Ewing's sarcoma. EWS-FLI1 fusion was evaluated. Recent advances in understanding the EWS-FLI1 fusion in Ewing's sarcoma reveal its mitogenic targets, role in splicing deregulation, and modulation by the p53 pathway.
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