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Genetic and Rare Diseases Information Center (GARD)

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Synovial sarcoma


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Overview



What is synovial sarcoma?

What are the signs and symptoms of synovial sarcoma?

What causes synovial sarcoma?

How is synovial sarcoma diagnosed?

How might synovial sarcoma be treated?


What is synovial sarcoma?

Synovial sarcoma is rare and aggressive soft tissue sarcoma that appears as deep, painless, slowly growing masses. Most frequently the tumor affects the legs, especially the knees, and the arms. Less frequently, this disease develops in the trunk, head and neck region, or the abdomen. Although synovial sarcoma does not have a clearly defined cause, genetic factors are believed to influence the development of this disease. Cells in these tumors are usually characterized by the presence of a translocation involving chromosomes X and 18. This translocation is specific to synovial sarcoma and is often used to diagnose this cancer. Although this condition generally affects adults, about 30% of reported cases occur in children and adolescents.[1][2]
Last updated: 7/11/2011

What are the signs and symptoms of synovial sarcoma?

Synovial sarcoma is a slow-growing tumor. Because it grows slowly, a person may not have or notice symptoms for some time, resulting in a delay in diagnosis. The most common symptoms of are swelling or a mass that may be tender or painful. The tumor may limit range of motion or press against nerves and cause numbness. The symptoms of synovial sarcoma can be mistaken for those of inflammation of the joints (arthritis) or other non-cancerous conditions that cause inflammation such as bursitis and synovitis.[1]
Last updated: 7/11/2011

What causes synovial sarcoma?

The exact cause of synovial sarcoma is not entirely understood; however, studies have indicated that genetic alterations may play a role in the formation of soft tissue sarcomas. In synovial sarcoma, a rearrangement in the chromosome material between chromosomes X and 18 is usually present. The characteristic chromosome translocation  t(X;18)(p11.2;q11.2) is present in nearly all synovial sarcomas. This rearrangement changes the position and function of genes, causing a fusion of genes referred to as a "fusion transcript." Patients have an abnormal fusion transcript involving two genes which creates a novel (new) gene. This important discovery has led to improvements in diagnosing this condition, and may lead to newer treatments in the future.[3]
Last updated: 7/11/2011

How is synovial sarcoma diagnosed?

A doctor may use the following procedures and tests to diagnose synovial sarcoma:[1]
  • Biopsy: Tissue is removed for examination under a microscope.
  • Immunohistochemical analysis: Tumor tissue is tested for certain antigen and antibody interactions common to synovial sarcoma.
  • Ultrastructural findings: The tissue is examined using an ultramicroscope and electron microscope.
  • Genetic testing: Tissue is tested for a specific chromosome abnormality common to synovial sarcoma. The characteristic chromosome translocation t(X;18)(p11.2;q11.2) is present in nearly all synovial sarcomas, providing strong evidence for the diagnosis of this tumor.[2]
Last updated: 7/11/2011

How might synovial sarcoma be treated?

The type of treatment depends on the age of the person, the location of the tumor, its size, its grade (how abnormal the cancer cells look under a microscope and how likely the tumor will quickly grow and spread), and the extent of the disease. The most common treatment is surgery to remove the entire tumor with negative margins (no cancer cells are found at the edge or border of the tissue removed during surgery). If the first surgery does not obtain negative tissue margins, a second surgery may be needed.[1]
Radiation therapy before or after surgery may also be necessary to control the tumor or decrease the chance of recurrence (cancer coming back). The use of intraoperative radiation therapy (radiation aimed directly at the tumor during surgery) and brachytherapy (radioactive material sealed in needles, wires, seeds, or catheters, and placed directly into or near a tumor) are under study. Affected individuals may also receive chemotherapy alone or in combination with radiation therapy.[1]
Last updated: 7/11/2011

References
  1. Synovial Sarcoma: Questions and Answers. National Cancer Institute (NCI). 2005; http://www.cancer.gov/cancertopics/factsheet/Sites-Types/synovial. Accessed 7/11/2011.
  2. Ruggiero A. Synovialsarcoma. Orphanet. March 2004; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=3273. Accessed 7/11/2011.
  3. Synovial sarcoma. Children's Hospital Boston. http://www.childrenshospital.org/az/Site1031/mainpageS1031P1.html. Accessed 7/11/2011.