eMedicine Specialties > Orthopedic Surgery > Neoplasms

Synovial Cell Sarcoma: Treatment

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

Treatment

Medical Therapy

Adjuvant chemotherapy and neoadjuvant chemotherapy have been proposed for patients with metastatic soft tissue sarcomas. Nevertheless, chemotherapy in the treatment of this sarcoma remains controversial. Ladenstein et al have reported improved survival rates with the use of adjuvant doxorubicin- and cyclophosphamide-based chemotherapy. Other authors have recommended combinations of  doxorubicin (75 mg/m2 via continuous infusion over 3 days) and bolus ifosfamide (2.5 g/m2 daily for 4 days, or ifosfamide with liposomal daunorubicin). Granulocyte colony-stimulating factor may stimulate the bone marrow. Chemotherapy should be considered in patients with extremity tumors greater than 5 cm.1,2,3,7

Some studies have shown promising results in the treatment of synovial cell sarcoma xenografts with a murine monoclonal antibody.16 This monoclonal antibody attacks a frizzled homologue called FZD10 (a cell-surface receptor), which is present in the synovial sarcoma cells and absent in the normal organs.  Clinical applications of these monoclonal antibodies are not still available.  An additional innovative technique could be a SYT-SSX–derived peptide vaccine.11

Another controversial aspect of treatment of synovial cell sarcoma is the efficacy of chemotherapy as adjuvant treatment after surgery. Chemotherapy has not proved to provide a significant benefit in survival rates in all series. A multicenter clinical trial for the treatment of patients with soft tissue sarcoma includes patients with soft tissue sarcoma stage IV and evaluates the clinical response of a treatment with topotecan and carboplatin. Information is available on the clinical trial web site. 

Surgical Therapy

Surgical excision is still the cornerstone of treatment for synovial cell sarcoma. A tumor-free margin of 1–3 cm is recommended.4 Maximal care must be taken to reduce the risk of local recurrence. As the tumor extends along fascial planes, careful preoperative planning with MRI is necessary before embarking on a wide excision. Because of the tumor's predilection for the popliteal fossa, limb salvage may not be possible because of the proximity of the neurovascular structures. Even with microscopically negative margins, patients could develop local recurrence. Rare local recurrence has been reported 15 years after the initial treatment.9 Surgical resection of isolated metastases may be possible if the tumor is well controlled. Palliative surgery may also be appropriate, particularly to alleviate pain or achieve hemorrhage control.17

Preoperative Details

Staging prior to surgery is essential, as synovial cell sarcoma spreads along fascial planes. Radiologic evaluation before treatment is very important. Imaging techniques employed in preoperative evaluation include radiographs, MRI, and CT scans.

Synovial cell sarcoma has the ability to metastasize via the lymphatic system.18 Venous metastasis can occur as well. Synovial sarcoma is most likely to invade adjacent bone.

Preoperative radiation therapy is associated with an increased rate of wound problems. This neoadjuvant radiotherapy is sometimes proposed before surgery to reduce the size of the tumor.

Intraoperative Details

The ideal surgical approach takes into account the location of the lesion and must always include the possibility of amputation in cases of unsuccessful total resection. Thus, a radical or wide resection is indicated, depending on the location of the tumor. The mass may be tagged so that the location of any close or contaminated margins can be identified. A primary amputation is proposed if the location and extension of the tumor do not provide adequate function of the extremity. Primary amputation is required in 20% of patients.1 Vascular resection and reconstruction are most often performed in the lower extremities.19

Postoperative Details

Postoperative radiation therapy is usually required, particularly if the margins are close to vital neurovascular structures. The most common radiotherapy is external-beam radiation directed at the tumor site, including a margin of surrounding normal tissue. The decision about the timing of radiation therapy (ie, before or after surgery) is controversial.

Local radiation is usually 40-60 Gy. Vital neurologic structures, open physes, or an extreme peripheral location (hand or foot) can make external-beam radiation therapy potentially hazardous. Brachytherapy (radiation administered by a local implant) is an alternative consideration.20 Intensity-modulated radiation therapy (IMRT) has also been proposed.1

Follow-up

Follow-up involves clinical examination, MRI of the surgical site, and CT scan of the chest. After surgical treatment, the authors recommend an MRI, a CT scan, and patient review every 3-6 months for the first 2 years and then every 6 months for the next 3 years. Most recurrent metastatic disease occurs within the first 2 years, but late recurrence has been documented.21

Complications

The surgical complications are related to the site involved but include the general complications of wound infection, wound breakdown, neurologic or vascular injury, and hematoma or seroma formation.

Specific complications associated with this tumor are local recurrence and distant metastases. The risk of local recurrence is directly proportional to the adequacy of surgical clearance. Therefore, a wide excision is mandatory to reduce this risk. Essential neurologic structures may make a wide clearance impossible, especially in the popliteal fossa. Nerve grafting and/or later muscle transfers may need to be considered to allow the wide margin needed.

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].

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  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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  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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  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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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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