Synovial Sarcoma of the Foot
摘要
Synovial sarcoma (SS) is a relatively common neoplasm accounting for 5–10% of all soft tissue sarcomas, typically occurring in younger patients between 15 and 35 years, predominately near the large joints of the extremities (60–70% in the lower extremity). Contrary to its name, SS does not originate from the synovial cell, and although it arises from within the joint in less than 5% of all cases, it is considered to have the highest incidence among primary intra-articular malignancies. The exact cell of origin remains unknown. This disease is relatively rare, accounting for approximately 5–10% of all soft tissue sarcomas, and typically affects adolescents and young adults. Synovial sarcoma is often characterized by a specific chromosomal translocation (t(X;18)(p11;q11)), which results in the formation of an abnormal fusion gene, SS18-SSX. This gene is believed to play a critical role in the development of the disease, although the exact mechanisms are not yet fully understood. The fusion gene leads to abnormal cell growth and division, resulting in the formation of a tumor. It typically presents as a slow-growing mass that may be indolent, and it is the most common sarcoma of the foot. Imaging may show a soft tissue mass with sometimes presence of calcification, and on T2 magnetic resonance imaging (MRI) sequence, there is typically a heterogeneous “triple-signal” (i.e., high, intermediate, and low intensity). Histologically, it presents most frequently as a biphasic form containing both a spindle cell and epithelioid components but it may also occur as monophasic, i.e., spindle cell pattern only. Surgery is usually the first-line treatment, aiming to remove the entire tumor along with a margin of healthy tissue. Therefore, adjuvant therapies, such as radiation therapy and chemotherapy, are often used to enhance the effectiveness of surgery and reduce the risk of recurrence. Factors that influence the prognosis include the size and location of the tumor, the patient’s age and general health, and the extent of tumor spread at the time of diagnosis. The 5-year survival rate is reported to range from 30% to 74%.