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Genetic and Rare Diseases Information Center (GARD)

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Synovial sarcoma


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Your Question

I need to know more information about synovial sarcoma. My aunt was diagnosed with this condition, which showed up in her kidney and then spread all over her abdominal area. I want to understand more about it in terms of genetics and the outcome of patients. I can't find much research since it is so rare and any alternative treatments.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

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

What is the prognosis for synovial sarcoma?

The prognosis depends on various factors, including the size of the tumor, invasiveness, grade, post-surgical margins, and the presence of metastases (cancer spread to other areas of the body). It is common for synovial cancer to recur (come back), usually within the first two years after treatment. Half of the cases of synovial sarcoma metastasize (spread) to the lungs, lymph nodes, or bone marrow.[1]
Last updated: 7/11/2011

How can I learn about current treatment research involving synovial sarcoma?

Participation in clinical trials is an important treatment option for many people with synovial sarcoma. Studies are in progress to determine the effectiveness of biological therapies (treatment to stimulate or restore the ability of the immune system to fight cancer), including monoclonal antibodies, and chemotherapy with hyperthermia (kills tumor cells by heating them to several degrees above body temperature).[1]

You can obtain information about clinica trials by calling the National Cancer Institute Cancer Information Service (CIS) toll-free at 1-800-4-CANCER (1-800-422-6237). CIS provides the most current information on cancer for patients, health professionals, and the general public.

NCI Public Inquiries Office
6116 Executive Blvd., Room 3036A
Bethesda, MD 20892-8322
Toll free: 1-800-422-6237
TTY: 800-332-8615
Online form: http://www.cancer.gov/global/contact/email-us
Web site: http://www.cancer.gov/aboutnci/cis

Additionally, the U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. Currently, 16 clinical trials are identified as enrolling individuals with synovial sarcoma. To find these trials, click here. After you click on a study, review its 'eligibility' criteria to determine its appropriateness. Use the study’s contact information to learn more. Check this site often for regular updates.

You can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH). We recommend calling the toll-free number listed below to speak with a specialist, who can help you determine if you are eligible for any clinical trials.

Patient Recruitment and Public Liaison Office
NIH Clinical Center
Bethesda, Maryland 20892-2655
Toll-free: 800-411-1222
Fax: 301-480-9793
E-mail: prpl@mail.cc.nih.gov
Web site:  http://clinicalcenter.nih.gov/

If you are interested in enrolling in a clinical trial, you can find helpful general information on clinical trials at the following ClinicalTrials.gov Web page.
http://clinicaltrials.gov/ct2/info/understand

Resources on many charitable or special-fare flights to research and treatment sites and low-cost hospitality accommodations for outpatients and family members, as well as ambulance services, are listed on the Web site of the Office of Rare Diseases Research (ORDR), part of the National Institutes of Health.
http://rarediseases.info.nih.gov/Resources.aspx?PageID=8
Last updated: 7/11/2011

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