The Biology of Synovial Sarcoma: State-of-the-Art and Future Perspectives

Abstract

New molecular insights are being achieved in synovial sarcoma (SS) that can provide new potential diagnostic and prognostic markers as well as therapeutic targets. In particular, the advancement of research on epigenomics and gene regulation is promising. The concrete hypothesis that the pathogenesis of SS might mainly depend on the disruption of the balance of the complex interaction between epigenomic regulatory complexes and the consequences on gene expression opens interesting new perspectives. The standard of care for primary SS is wide surgical resection combined with radiation in selected cases. The role of chemotherapy is still under refinement and can be considered in patients at high risk of metastasis or in those with advanced disease. Cytotoxic chemotherapy (anthracyclines, ifosfamide, trabectedin, and pazopanib) is the treatment of choice, despite several possible side effects. Many possible drug-able targets have been identified. However, the impact of these strategies in improving SS outcome is still limited, thus making current and future research strongly needed to improve the survival of patients with SS.

Keywords: Sarcoma epigenomics; Synovial sarcoma diagnosis; Synovial sarcoma genomics; Synovial sarcoma treatment.

Fig. 1

T2w axial MRI of the left thigh in a 54-year-old male, affected by synovial sarcoma with multiple lung metastasis at diagnosis, showed a large inhomogeneous mass with the so-called “triple sign”: fibrotic areas (low signal intensity — arrow), solid cellular elements (intermediate signal — arrowhead), and hemorrhage/necrosis areas (high signal intensity — asterisks)

Fig. 2

Conventional radiograph (left) and T2w axial fat-saturated MRI (right) of the left thigh in 32-year-old female with synovial sarcoma, showed calcific area (arrow), and the so-called “bowl of grapes” appearance with rounded cystic necrotic/hemorrhagic areas (arrowheads), containing a large fluid–fluid level (dotted arrow)