Alma Mater Studiorum - University of Bologna, Bologna, Italy.
IRCCS Azienda Ospedaliero Universitaria di Bologna, via Massarenti 9, 40138, Bologna, Italy.
Curr Treat Options Oncol. 2021 Oct 23;22(12):109. doi: 10.1007/s11864-021-00914-4.
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.
滑膜肉瘤（SS）的分子研究取得了新的进展，为其提供了新的潜在诊断和预后标志物以及治疗靶点。特别是，表观遗传学和基因调控研究的进展前景广阔。滑膜肉瘤的发病机制可能主要依赖于表观基因组调控复合物之间的复杂相互作用的平衡被打破，以及对基因表达的影响，这一具体假设为我们提供了有趣的新视角。对于原发性 SS，标准的治疗方法是广泛的手术切除，在某些情况下结合放疗。化疗的作用仍在进一步完善中，可以考虑用于有高转移风险的患者或晚期疾病患者。细胞毒性化疗（蒽环类药物、异环磷酰胺、曲贝替定和帕唑帕尼）是首选的治疗方法，尽管可能有一些副作用。已经确定了许多可能的药物靶点。然而，这些策略在改善 SS 预后方面的效果仍然有限，因此目前和未来的研究强烈需要提高 SS 患者的生存率。
