eMedicine Specialties > Orthopedic Surgery > Neoplasms

Synovial Cell Sarcoma

Author: Bernardo Vargas, MD, Consulting Staff, Department of Orthopedic Surgery, Hôpital Universitaire de Geneva, Switzerland
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
Contributor Information and Disclosures

Updated: Aug 7, 2008

Introduction

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

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.

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

Etiology

Synovial cell sarcoma is characterized by a specific chromosomal translocation t(X;18)(p11;q11). 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.5,8,9,10,11

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.5,8,9,10,11

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.12 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.

Relevant Anatomy

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

  • Truncal location involves the head, 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.

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.

More on Synovial Cell Sarcoma

Overview: Synovial Cell Sarcoma
Workup: Synovial Cell Sarcoma
Treatment: Synovial Cell Sarcoma
Follow-up: Synovial Cell Sarcoma
Multimedia: Synovial Cell Sarcoma
References
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References

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  2. Spira AI, Ettinger DS. The use of chemotherapy in soft-tissue sarcomas. Oncologist. 2002;7:348 –359. [Medline].

  3. Siehl J., Thiel E. Schmittel A. et al. Ifosfamide/Liposomal Daunorubicin Is a Well Tolerated and Active First-Line Chemotherapy Regimen in Advanced Soft Tissue Sarcoma Cancer. 2005;104 .3:611-617. [Medline].

  4. Zagard G. Ballo M., Pisters P.et al. Prognostic factors for patients with localized soft tissue sarcoma treated with conservation surgery and radiation therapy. Cancer. 2003;97. 10:2530-2543. [Medline].

  5. Ladanyi M, Antonescu CR, Leung DH, and als. Impact of SYT-SSX fusion type on the clinical behavior of synovial sarcoma: a multi-institutional retrospective study of 243 patients. Cancer Res. 2002;62(1):135-40. [Medline].

  6. Deshmukh R., Mankin, H. J. Singer S. Synovial Sarcoma: The Importance of Size and Location for Survival Clin Orthop 2004 : 419, 155-161. [Medline].

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  8. Antonescu CR, Kawai A, Leung DH, Lonardo F, Woodruff JM, Healey JH, et al. Strong association of SYT-SSX fusion type and morphologic epithelial differentiation in synovial sarcoma. Diagn Mol Pathol. 2000;9(1):1-8. [Medline].

  9. Guillou L, Coindre JM, Gallagher G, et al:. Detection of the synovial sarcoma translocation t(X;18) (SYT-SSX) in paraffin-embedded tissues using reverse transcriptase-polymerase chain reaction: A reliable and powerful diagnostic tool for pathologists—A molecular analysis of 221 mesenchymal tumors fixed in different fixatives. Hum Pathol. 2001;32:105-112. [Medline].

  10. Kawai A, Woodruff J, Healey JH, et al. SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. N Engl J Med. Jan 15 1998;338(3):153-60. [Medline].

  11. Kawaguchi S, Wada T, Ida K, et al.:. Phase I vaccination trial of SYT-SSX junction peptide in patients with disseminated synovialsarcoma. J Transl Med. 2005;3:1. [Medline].

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  13. Bridge JA. Cytogenetic and molecular cytogenetic techniques in orthopaedic surgery. J Bone Joint Surg Am. Apr 1993;75(4):606-14. [Medline].

  14. Ten Heuvel SE, Hoekstra HJ, Suurmeijer AJ. Diagnostic Accuracy of FISH and RT-PCR in 50 Routinely Processed Synovial Sarcomas. Appl Immunohistochem Mol Morphol. Feb 8; [Epub ahead of print] 2008;[Medline].

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  16. Fukukawa C., Hanaoka H., Nagayama S. and als. Radioimmunotherapy of human synovial sarcoma using a monoclonal antibody against FZD10. Cancer Sci. 2008;99, 2 :432–440. [Medline].

  17. Sakabe T, Murata H, Konishi E, Takeshita H, Ueda H, Matsui T, et al. Evaluation of clinical outcomes and prognostic factors for synovial sarcoma arising from the extremities. Med Sci Monit. Jun 2008;14(6):CR305-310. [Medline].

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  19. Guert M., Abudu A., Driver N., and als. The Indications for and the Prognostic Significance of Amputation as the Primary Surgical Procedure for Localized Soft Tissue Sarcoma of the Extremity. Annals of Surgical Oncology,. 2004.;12(1):10-17. [Medline].

  20. Pisters PWT, Harrison LB, Leung DHY, et al:. Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. J Clin Oncol. 1996;14:859-868. [Medline].

  21. Moreno Martín-Retortillo L, Andrés Moreno MM, Cañete Nieto A, Castel Sánchez V. Synovial sarcoma in children. A single centre experience. Clin Transl Oncol. Jul 2007;9(7):468-70. [Medline].

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Further Reading

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Keywords

synovial cell sarcoma, synovial sarcoma, synovial cell, connective tissue tumor, connective tissue neoplasm, synovioma, malignant neoplasm, malignant tumor, joint tumors

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Contributor Information and Disclosures

Author

Bernardo Vargas, MD, Consulting Staff, Department of Orthopedic Surgery, Hôpital Universitaire de 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

Medical Editor

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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.

CME Editor

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, American Association of Physicians of Indian Origin, American College of International Physicians, and American College of 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 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.

 
 
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