Department of Surgery, Division of Orthopaedic Surgery, McMaster University, Hamilton, ON L8V 1C3, Canada.
Department of Pathology and Molecular Medicine, McMaster University, Hamilton, ON L8S 4L8, Canada.
Curr Oncol. 2021 May 19;28(3):1909-1920. doi: 10.3390/curroncol28030177.
Synovial sarcomas (SS) represent a unique subset of soft tissue sarcomas (STS) and account for 5-10% of all STS. Synovial sarcoma differs from other STS by the relatively young age at diagnosis and clinical presentation. Synovial sarcomas have unique genomic characteristics and are driven by a pathognomonic t(X;18) chromosomal translocation and subsequent formation of the SS18:SSX fusion oncogenes. Similar to other STS, diagnosis can be obtained from a combination of history, physical examination, magnetic resonance imaging, biopsy and subsequent pathology, immunohistochemistry and molecular analysis. Increasing size, age and tumor grade have been demonstrated to be negative predictive factors for both local disease recurrence and metastasis. Wide surgical excision remains the standard of care for definitive treatment with adjuvant radiation utilized for larger and deeper lesions. There remains controversy surrounding the role of chemotherapy in the treatment of SS and there appears to be survival benefit in certain populations. As the understanding of the molecular and immunologic characteristics of SS evolve, several potential systematic therapies have been proposed.
滑膜肉瘤（SS）是一种独特的软组织肉瘤（STS）亚型，占所有 STS 的 5-10%。滑膜肉瘤与其他 STS 的区别在于其发病年龄和临床表现相对年轻。滑膜肉瘤具有独特的基因组特征，由特征性的 X;18 染色体易位和随后形成的 SS18:SSX 融合癌基因驱动。与其他 STS 类似，诊断可以通过病史、体格检查、磁共振成像、活检和随后的病理学、免疫组织化学和分子分析的结合获得。肿瘤大小增加、年龄和肿瘤分级已被证明是局部疾病复发和转移的阴性预测因素。广泛的手术切除仍然是明确治疗的标准，对于较大和较深的病变，辅助放疗是必要的。化疗在 SS 治疗中的作用仍存在争议，在某些人群中似乎有生存获益。随着对 SS 的分子和免疫特征的理解不断发展，已经提出了几种潜在的系统治疗方法。
