The genetic characterization of DSRCT is a chromosomal translocation of t(11;22)(p13;q12) between Ewing's sarcoma (EWS) gene on chromosome 22 and Wilm's tumor (WT1) gene on chromosome 11, leading to a EWS-WT1 fusion transcript; the characteristic translocation t(11;22)(p13;q12) is specific for DSRCT, regardless of its site [11, 16]. This evidence concerns the gene EWSR1 and Ewing sarcoma.