What is Synovial Sarcoma?

 

Synovial sarcoma (SS) is a rare sarcoma driven by a translocation between SS18 and SSX 1, 2, or 4. The hybrid transcription factor SS18:SSX alters SWItch/Sucrose Non-Fermentable (SWI/SNF) chromatin remodeling and global methylation patterns that may allow future therapeutic opportunities.

Current treatment of Synovial Sarcoma involves multimodal approach, incorporating surgery, chemotherapy, and radiotherapy. Currently, surgery remains the mainstay for treatment of synovial sarcoma. While considering the overall disease management perspective, first-line treatment usually consists of wide margin surgery plus radiotherapy. However, DelveInsight’s market forecast model is based entirely on pharmacological therapies and does not include any procedural or surgical methods.

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As per the latest treatment paradigms, chemotherapy regimen is administered for a fixed number of cycles followed by a watchful waiting approach to assess the optimum response. Doxorubicin and ifosfamide constitute the first-line approach generating a large segment of synovial sarcoma’s chemotherapeutic market.

Doxorubicin with or without ifosfamide remains the mainstay of first-line chemotherapy for synovial sarcoma treatment although data from recent studies suggest that SS patients might benefit from first-line combination treatment of ifosfamide with doxorubicin over monotherapy doxorubicin. However, due to rarity of disease, this cannot be considered a final conclusive result. 

Compared with doxorubicin, ifosfamide has the advantage of having no dose-limiting cumulative toxicity. Also, given that it shows activity in SS patients, ifosfamide (in high doses) is the favored second-line treatment option. Other alternative options for second-line and beyond include Yondelis (Trabectedin) and Pazopanib (Votrient) which are administered when patients relapse or fail to respond to first-line treatment options.

Trabectedin (Yondelis), an antitumor agent is approved in Europe for the treatment of adult patients with advanced soft tissue sarcoma after failure of anthracyclines (doxorubicin) and ifosfamide, and also for those who are unfit to receive these agents, whereas in the US, it has been approved for specific types of Soft Tissue Sarcoma, but its utility as a therapeutic for SS treatment is uncertain.

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