Synovial Sarcoma: A Clinical Review

Abstract

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.

Malignant primary intraosseus synovial sarcoma – a rare case report

“…Wide surgical resection is the standard of care for management of this lesion with adjuvant radiation therapy for deeper lesions and chemotherapy showing some benefits in certain populations although it remains controversial [ 5 , 17 ]. We performed below elbow amputation and in conjunction with adjuvant radio- and chemotherapy, we therefore expect a marked increase in life expectancy.…”

Biphasic synovial sarcomas of the liver: a case report and literature review

“…Microscopically, biphasic hepatic synovial sarcoma is composed of different proportions of both spindle cells and epithelioid cells, of which the spindle cells are often uniform in size and tightly arranged in a striated, fasciculated or herringbone shape. Epithelioid cells are often cubic or tall columnar in shape and may be arranged in a glandular, nest-like or papillary structure [ 1 , 10 ]. Immunohistochemistry is crucial in the diagnosis of hepatic synovial sarcoma.…”

“…Approximately 90% of synovial sarcomas are positive for keratin, and approximately 60% of cases will be positive for CD99. Additionally, CD34 is negative in almost all synovial sarcomas [ 1 , 10 ]. Other common pathological manifestations include vimentin (+), S-100 (−), DES (−), SMA (−) and TLE1 (+) [ 5 ], all of which are consistent with the immunohistochemical findings of the patient in this case.…”

“…Synovial sarcoma (SS) is a rare but highly malignant soft tissue sarcoma, accounting for approximately 5–10% of soft tissue sarcomas [ 1 ]. However, it does not originate in the synovium, and most current studies suggest that it may originate from primitive mesenchymal cells in mesenchymal tissue [ 2 ].…”

“…However, it does not originate in the synovium, and most current studies suggest that it may originate from primitive mesenchymal cells in mesenchymal tissue [ 2 ]. The most important feature of synovial sarcoma is the ability to biphasically differentiate into the epithelium and mesenchyme, which can be divided into three subtypes: biphasic, unidirectional and hypodifferentiated [ 1 ]. It most frequently occurs in the large joints and surrounding soft tissues of the extremities.…”