Synovial Sarcoma Lucy VanGieson July 29, 2004 What is Synovial Sarcoma? This is a rare and aggressive soft tissue malignant tumor that begins near joints in the body. Synovial sarcomas are found in tendons, bursae (fluid-filled, cushioning sacs located in the spaces between tendons, ligaments and bones), and in the hollow areas that separates the bones of a joint. Tumor Sites These tumors occur mainly in the legs and arms where they tend to arise near large joints, especially around the knee. The most common location of origin of Synovial sarcomas are in the thigh near the knee. Rarely this cancer will form in the head and neck or in the trunk. Who is Affected? Synovial sarcomas occur more frequently in older adolescents and young adults, although it can present itself at any age. It is reported that the mean age of patients is 30 years. Synovial sarcomas affect slightly more men than women. Statistics Synovial sarcomas account for approximately 10% of all human malignant sarcomas. 30% of all reported cases manifest themselves during the first two decades of life. Approximately 800 new cases occur per year in the United States. Risk Factors There are no known risk factors for developing this form of cancer. Symptoms The most common symptom experienced is a swelling or mass that may be accompanied by pain or tenderness. In a few cases it has been noted that pain or tenderness was present for several years even though a mass could not be felt. Limping or difficulty using legs, arms, hands or feet have been reported. Etiology The exact etiology of Synovial sarcomas is unknown. What is known is that many Synovial sarcoma cells are characterized by a chromosomal translocation that fuses the SYT gene from chromosome 18 to either the SSX1 or the SSX2 gene. Etiology Continued The fusion proteins SYT-SSX1 and SYT- SSX2 are believed to function as abnormal transcriptional regulators that result in either activation of proto-oncogenes or inhibition of tumor suppressor genes. Diagnostic Methods The most effective way to diagnose this cancer is by having a tissue biopsy. A Magnetic Resonance Imaging (MRI) is very useful in determining the extent to which the cancer has grown. Lastly, about one-third of Synovial sarcomas have been diagnosed using a Computed Tomography (CT) Scan. Treatments Synovial sarcomas may be treated by using the following medical approaches… Surgery Radiation Chemotherapy Surgery Complete surgical excision of the tumor, nearby muscle and lymph nodes is the best way of treating this cancer. Depending on the location and size of the tumor, it may be necessary to remove all or part of a limb. Radiation Radiation is often used in conjunction with surgery to kill cancer cells. It can be given before surgery in order to shrink a tumor or afterwards to kill any remaining cancer cells. On rare occasions radiation alone has been used for treatment of the primary tumor. Chemotherapy Chemotherapy has been proven highly effective with treating this form of cancer and it may be administered in one of the following ways… As a pill to swallow As an injection into the muscle or fat tissue Intravenously is the most common form of delivery for chemotherapy drugs. Prognosis Synovial sarcoma has a survival rate of approximately 50% at five years. When chemotherapy and radiation have been used as adjuvant therapy the survival rate increases to 73% at five years. Recurrence of the disease has been reported up to ten years following treatment and the survival rate significantly drops to less than 25%. Conclusion Although the occurrence of Synovial sarcomas are rare they can be locally aggressive and should be treated promptly. Once a patient has been diagnosed they must have continuous follow-up care for the rest of their lives. Patients with Synovial sarcoma are encouraged to join clinical trials since little is known about this cancer. Questions?
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