Diagnosis and management of synovial sarcoma.
Synovial sarcoma is a unique tumor with substantial promise for biologically targeted therapy. Although it demonstrates moderate chemosensitivity, with approximately 50% response rates to ifosfamide- and doxorubicin-containing regimens, it has a diagnostic translocation and a potentially informative chimeric protein product. Although surgical management remains the cornerstone to effect local control, therapeutic advancements are unlikely to occur by continuing to include advanced cases of synovial sarcomas in trials with other soft tissue sarcomas. Rather, attention should be turned toward prospective molecular targets and development of novel agents to exploit them. Research should be directed at understanding the fusion protein of the X,18 translocation and further validating the role of overexpressed proteins in synovial sarcoma. Meanwhile, carefully designed clinical trials of these agents, with translational correlates, will provide in vivo data to complement the preclinical experience.
Randall
R Lor
RL
Sarcoma Services, Suite 4260, Huntsman Cancer Institute, 2000 Circle of Hope, Salt Lake City, UT 84112, USA. r.lor.randall@lsc.utah.edu
Schabel
Kathryn L S
KL
Hitchcock
Ying
Y
Joyner
David E
DE
Albritton
Karen H
KH
D000293:Adolescent; D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000378:metabolism; D003131:Combined Modality Therapy; D004317:Doxorubicin / Q000627:therapeutic use; D006801:Humans; D007069:Ifosfamide / Q000627:therapeutic use; D015514:Oncogene Proteins, Fusion / Q000378:metabolism; D011379:Prognosis; D011878:Radiotherapy; D013584:Sarcoma, Synovial / Q000175:diagnosis / Q000378:metabolism / Q000628:therapy; D014178:Translocation, Genetic
D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor; D015514:Oncogene Proteins, Fusion; C116512:SYT-SSX fusion protein; D004317:Doxorubicin; D007069:Ifosfamide