The EWS gene spans approximately 40 kb of DNA and is composed of 17 exons [29]. The first seven exons encode the N-terminal region, which is involved in regulating the specificity of the RNA-binding capability of EWS [30]. The RNA-binding capability is localized to the 86 amino acids that comprise the C-terminal region [30]. Three key features indicate that this protein is encoded by a housekeeping gene: EWS is expressed ubiquitously, its expression is stable throughout the cell cycle, and its mRNA has a long half-life [31]. EWS belongs to a subgroup of RNA-binding proteins called the TET family, which also includes TLS/FUS and hTAFII68 [31,32]. Although more remains to be known about the role of native EWS, it is a nuclear protein that appears to be recruited to promoter regions where it associates with other factors to act as a promoter-specific transactivator [33]. It is noteworthy that TET family members are associated with nearly half of sarcomas with characteristic non-random chromosomal translocations, including ESFT, clear cell sarcoma, desmoplastic small round cell tumor, myxoid chondrosarcoma, and myxoid liposarcoma [34]. Interestingly, rare cases of tumors with ESFT-like features harboring FUS-ERG fusion transcripts have recently been reported [