The SYT-SSX1 and SYT-SSX2 fusion genes resulting from the chromosomal translocation t(X;18)(p11;q11) are chararcteristically found in synovial sarcoma.
Synovial sarcoma occurs most commonly in patients 15 to 40 years of age and affects males greater than females. It is a slow growing tumor that has a propensity for the juxta-articular regions around the knee, shoulder, arm, elbow, and foot. The treatment for synovial sarcoma is wide resection and radiation +/- chemotherapy with a 10 year survival of approximately 25%.
2-BCR-ABL is an oncogene associated with chronic myelogenous leukemia (CML), the result of a reciprocal translocation between chromosome 9 and 22.
3-The EWS (EWSR1) gene is involved in translocations in Ewing's sarcoma, clear cell sarcoma, desmoplastic small round cell tumor and myxoid liposarcoma. The Ewing sarcoma family of tumors is characterized by recurrent translocations that fuse EWS to one of the following genes FLI1 (>90% of cases), ERG, ETV1, E1AF and FEV.
4-PAX3-FKHR is a gene fusion seen in Alveolar Rhabdomyosarcoma and is specific for the high risk metastatic subgroup.
5-In clear cell sarcoma, DSRCT and myxoid liposarcoma EWS is fused to ATF1, WT1 and CHOP respectively.
Fletcher J: Cytogenic analysis of soft tissue tumors, in Soft Tissue Tumors, 2001; Kawai et al, N Engl J Med, 1998