Synovial Cell Sarcoma 

  • Author: Bernardo Vargas; Chief Editor: Harris Gellman, MD   more...
 
Updated: May 15, 2012
 

Background

Synovial cell sarcoma is one of the most common soft tissue tumors in adolescents and young patients, with approximately 1 in 3 cases occurring in the first 2 decades of life. Mean age of patients at diagnosis is approximately 30 years.

Prognosis of this relatively rare tumor is related to initial care. Survival rates have improved in the past 20 years because of treatment with primary radical surgery, along with chemotherapy and radiation.[1, 2, 3, 4]

The image below depicts a synovial sarcoma.

Lateral radiograph depicts a synovial sarcoma of tLateral radiograph depicts a synovial sarcoma of the dorsum of the hand. A small nodule, present for 5 years, rapidly enlarged to the present size over 2 months.
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Problem

The origin of synovial cell sarcoma is unclear. In contrast to its name, synovial cell sarcoma is not associated with synovial joints. Because of the similarity between cells of this tumor and primitive synoviocytes, the term synovial cell sarcoma has been used.

A neurologic origin has been suggested. In fact, there is a histologic resemblance between neural cells of malignant peripherical nerve sheath tumor (MPNST) and synovial cell sarcoma.[5] Typically, synovial cell sarcoma is associated with a history of a long-standing nodule, sometimes present for years, which increases rapidly in size over a few months; therefore, it is sometimes overlooked. The tumor spreads along fascial planes and, thus, can be much more widespread than apparent on initial evaluation.

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Epidemiology

Frequency

The incidence of synovial cell sarcoma has been estimated to be 2.75 per 100000.[6] The majority of cases involve the lower extremities. Approximately 800 new cases occur in the United States each year, and it represents around 5-10% of all soft tissue sarcomas. Synovial cell sarcoma is the third most common soft tissue tumor in adolescent and young adults.[7]

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Etiology

Synovial cell sarcoma is characterized by a specific chromosomal translocation t(X;18)(p11;q11).[8] This defect appears to be the underlying cause of the tumor. This specific chromosomal translocation between chromosome X and chromosome 18 has been noted in more than 90% of cases. This fusion gene is called, in genetic terms, the SYT-SSX1, SYT-SSX2, or SYT-SSX4. These terms correspond to a fusion of the SYT gene (chromosome 18) with the SSX gene (chromosome X). Females are more commonly affected than males in both SYT-SSX2 and SYT-SSX1 types. This association is stronger in SYT-SSX2. To our knowledge, the origin of this translocation has not been identified.[5, 9, 10, 11, 12, 13]

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Pathophysiology

The (X;18)(p11;q11) translocation fuses the SYT gene from chromosome 18 to either of 2 homologous genes at Xp11, either SSX1, SSX2, or SSX4. The fusion proteins SYT-SSX1 and SYT-SSX2 are believed to function as aberrant transcriptional regulators, resulting in either activation of proto-oncogenes or inhibition of tumor suppressor genes. A correlation appears to exist between the histologic subtype of the tumor and either of the 2 fusion proteins. Biphasic tumors, containing both epithelial and spindle cell components, express the SYT-SSX1 transcript, while monophasic tumors with only a spindle cell component may express either transcript.[5, 9, 10, 11, 12]

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Presentation

Synovial cell sarcoma usually occurs within the first 3 decades of life and generally is associated with a history of a small nodule that has increased rapidly in size.[14] The mass often is painful and deep. Most commonly, it is situated around the knee, but it also can appear in the hands and feet. Patients may show symptoms several months before their diagnosis.

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Relevant Anatomy

Survival analysis is correlated with location of the tumor in 3 anatomic regions:

  • Truncal location involves the head,[15] neck, thorax, abdomen, and pelvis.
  • Distal extremity involves the hands, feet, and ankles.
  • Proximal extremity involves the arms, forearms, thighs, and legs.

Distal extremity tumors have a better prognosis than proximal or truncal tumors.[6] Nevertheless, this malignancy can affect any part of the appendicular skeleton. Therefore, relevant anatomy depends on the site involved.

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Contraindications

There are no contraindications to surgery, as it is a potentially life-saving procedure. There is a relative contraindication to treat these patients in primary centers. Early referral to tertiary centers for definitive treatment must be preferred. Ideally, treatment should be performed by a multidisciplinary team with personnel experienced in the management of soft tissue sarcomas.

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Proceed to Workup
 
 
Contributor Information and Disclosures
Author

Bernardo Vargas  MD, Consulting Staff, Department of Orthopedic Surgery, University Hospital of Geneva, Switzerland

Disclosure: Nothing to disclose.

Coauthor(s)

Mark Clayer, MD, MBBS, FRACS, FAOrthA  Head of Musculoskeletal Tumor Service, Department of Orthopaedics and Trauma, Queen Elizabeth Hospital; Senior Visiting Medical Specialist, Royal Adelaide Hospital and Women's and Children's Hospital, Australia

Mark Clayer, MD, MBBS, FRACS, FAOrthA is a member of the following medical societies: Australian Medical Association and Australian Orthopaedic Association

Disclosure: Orthopedics hyperguide Honoraria Independent contractor; Stryker Grant/research funds Employment

Specialty Editor Board

Howard A Chansky, MD  Associate Professor, Department of Orthopedics and Sports Medicine, University of Washington Medical Center

Howard A Chansky, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Sean P Scully, MD, PhD  Professor, Department of Orthopedics, University of Miami

Sean P Scully, MD, PhD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, International Society on Thrombosis and Haemostasis, and Society of Surgical Oncology

Disclosure: Nothing to disclose.

Dinesh Patel, MD, FACS  Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital

Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD  Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami, Leonard M Miller School of Medicine

Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, and Arkansas Medical Society

Disclosure: Nothing to disclose.

References

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Lateral radiograph depicts a synovial sarcoma of the dorsum of the hand. A small nodule, present for 5 years, rapidly enlarged to the present size over 2 months.
T1-weighted MRI depicts a synovial sarcoma of the dorsum of the hand. The tumor has low signal on T1 weighting.
T2-weighted MRI depicts a synovial sarcoma of the dorsum of the hand. The tumor has a heterogeneous signal on T2 weighting, indicative of a variable growth pattern.
Although synovial sarcoma typically has a biphasic histology, this disease is often monophasic (lacking glandular differentiation), which produces the picture of a small, round blue cell tumor.
 
 
 
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