Skip to main content

Synovial Sarcoma

  • Chapter
  • First Online:
Management of Soft Tissue Sarcoma

Abstract

Synovial sarcomas present typically as a mass lesion in the extremities. In the authors’ experience, it is a soft tissue sarcoma that presents as a painful mass more commonly than other sarcomas. Historically thought to be associated with peripheral joints, it is clear that there is no association of this sarcoma with synovium per se. Clinical presentation is that of younger age groups than other sarcomas, predominantly a disease of adolescent and young adulthood. As our data set includes patients over age 16, we underemphasize the presence in adolescents (Fig. 8.1). Site distribution for this adult cohort is shown in Fig. 8.2. This histology stands to become an excellent proof of principle diagnosis for immunotherapy and epigenetic therapy in the coming few years.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

eBook
USD 16.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 129.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 199.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Haldar M, Hancock JD, Coffin CM, et al. A conditional mouse model of synovial sarcoma: insights into a myogenic origin. Cancer Cell. 2007;11:375–88.

    Article  CAS  PubMed  Google Scholar 

  2. van de Rijn M, Barr FG, Xiong QB, et al. Poorly differentiated synovial sarcoma: an analysis of clinical, pathologic, and molecular genetic features. Am J Surg Pathol. 1999;23:106–12.

    Article  PubMed  Google Scholar 

  3. Turc-Carel C, Dal Cin P, Limon J, et al. Involvement of chromosome X in primary cytogenetic change in human neoplasia: nonrandom translocation in synovial sarcoma. Proc Natl Acad Sci U S A. 1987;84:1981–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Lasota J, Jasinski M, Debiec-Rychter M, et al. Detection of the SYT-SSX fusion transcripts in formaldehyde-fixed, paraffin-embedded tissue: a reverse transcription polymerase chain reaction amplification assay useful in the diagnosis of synovial sarcoma. Mod Pathol. 1998;11:626–33.

    CAS  PubMed  Google Scholar 

  5. Skytting B, Nilsson G, Brodin B, et al. A novel fusion gene, SYT-SSX4, in synovial sarcoma. J Natl Cancer Inst. 1999;91:974–5.

    Article  CAS  PubMed  Google Scholar 

  6. Coindre JM, Pelmus M, Hostein I, et al. Should molecular testing be required for diagnosing synovial sarcoma? A prospective study of 204 cases. Cancer. 2003;98:2700–7.

    Article  PubMed  Google Scholar 

  7. 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. 1998;338:153–60.

    Article  CAS  PubMed  Google Scholar 

  8. Saito T, Nagai M, Ladanyi M. SYT-SSX1 and SYT-SSX2 interfere with repression of E-cadherin by snail and slug: a potential mechanism for aberrant mesenchymal to epithelial transition in human synovial sarcoma. Cancer Res. 2006;66:6919–27.

    Article  CAS  PubMed  Google Scholar 

  9. Saito T, Oda Y, Kawaguchi K, et al. E-cadherin mutation and Snail overexpression as alternative mechanisms of E-cadherin inactivation in synovial sarcoma. Oncogene. 2004;23:8629–38.

    Article  CAS  PubMed  Google Scholar 

  10. Saito T, Oda Y, Sugimachi K, et al. E-cadherin gene mutations frequently occur in synovial sarcoma as a determinant of histological features. Am J Pathol. 2001;159:2117–24.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Kadoch C, Crabtree GR. Reversible disruption of mSWI/SNF (BAF) complexes by the SS18-SSX oncogenic fusion in synovial sarcoma. Cell. 2013;153:71–85.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Robbins PF, Morgan RA, Feldman SA, et al. Tumor regression in patients with metastatic synovial cell sarcoma and melanoma using genetically engineered lymphocytes reactive with NY-ESO-1. J Clin Oncol. 2011;29:917–24.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Pervaiz N, Colterjohn N, Farrokhyar F, et al. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer. 2008;113:573–81.

    Article  PubMed  Google Scholar 

  14. Le Cesne A, Ouali M, Leahy MG, et al. Doxorubicin-based adjuvant chemotherapy in soft tissue sarcoma: pooled analysis of two STBSG-EORTC phase III clinical trials. Ann Oncol. 2014;25:2425–32.

    Article  PubMed  Google Scholar 

  15. Guadagnolo BA, Zagars GK, Ballo MT, et al. Long-term outcomes for synovial sarcoma treated with conservation surgery and radiotherapy. Int J Radiat Oncol Biol Phys. 2007;69:1173–80.

    Article  PubMed  Google Scholar 

  16. Folkert MR, Singer S, Brennan MF, et al. Comparison of local recurrence with conventional and intensity-modulated radiation therapy for primary soft-tissue sarcomas of the extremity. J Clin Oncol. 2014;32:3236–41.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Sarcoma Meta-Analysis Collaboration: Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Lancet 350:1647-54, 1997

    Google Scholar 

  18. Woll PJ, van Glabbeke M, Hohenberger P, et al: Adjuvant chemotherapy (CT) with doxorubicin and ifosfamide in resected soft tissue sarcoma (STS): Interim analysis of a randomised phase III trial. J Clin Oncol (Meeting Abstracts) 25:10008, 2007

    Google Scholar 

  19. Eilber FC, Brennan MF, Eilber FR, et al. Chemotherapy is associated with improved survival in adult patients with primary extremity synovial sarcoma. Ann Surg. 2007;246:105–13.

    Article  PubMed  PubMed Central  Google Scholar 

  20. Shiu MH, Hilaris BS, Harrison LB, et al. Brachytherapy and function-saving resection of soft tissue sarcoma arising in the limb. Int J Radiat Oncol Biol Phys. 1991;21:1485–92.

    Article  CAS  PubMed  Google Scholar 

  21. Nori D, Schupak K, Shiu MH, et al. Role of brachytherapy in recurrent extremity sarcoma in patients treated with prior surgery and irradiation. Int J Radiat Oncol Biol Phys. 1991;20:1229–33.

    Article  CAS  PubMed  Google Scholar 

  22. Eggermont AM, de Wilt JH, ten Hagen TL. Current uses of isolated limb perfusion in the clinic and a model system for new strategies. Lancet Oncol. 2003;4:429–37.

    Article  PubMed  Google Scholar 

  23. Eggermont AM, Schraffordt Koops H, Lienard D, et al. Isolated limb perfusion with high-dose tumor necrosis factor-alpha in combination with interferon-gamma and melphalan for nonresectable extremity soft tissue sarcomas: a multicenter trial. J Clin Oncol. 1996;14:2653–65.

    CAS  PubMed  Google Scholar 

  24. Grunhagen DJ, de Wilt JH, Graveland WJ, et al. The palliative value of tumor necrosis factor alpha-based isolated limb perfusion in patients with metastatic sarcoma and melanoma. Cancer. 2006;106:156–62.

    Article  PubMed  Google Scholar 

  25. Fayette J, Coquard IR, Alberti L, et al. ET-743: a novel agent with activity in soft tissue sarcomas. Oncologist. 2005;10:827–32.

    Article  CAS  PubMed  Google Scholar 

  26. Schoffski P, Ray-Coquard IL, Cioffi A, et al. Activity of eribulin mesylate in patients with soft-tissue sarcoma: a phase 2 study in four independent histological subtypes. Lancet Oncol. 2011;12:1045–52.

    Article  PubMed  Google Scholar 

  27. Sleijfer S, Ray-Coquard I, Papai Z, et al. Pazopanib, a multikinase angiogenesis inhibitor, in patients with relapsed or refractory advanced soft tissue sarcoma: a phase II study from the European organisation for research and treatment of cancer-soft tissue and bone sarcoma group (EORTC study 62043). J Clin Oncol. 2009;27:3126–32.

    Article  CAS  PubMed  Google Scholar 

  28. Maki RG, Jungbluth AA, Gnjatic S, et al. A Pilot Study of Anti-CTLA4 antibody ipilimumab in patients with synovial sarcoma. Sarcoma. 2013;2013:168145.

    Article  PubMed  PubMed Central  Google Scholar 

  29. Lubieniecka JM, de Bruijn DR, Su L, et al. Histone deacetylase inhibitors reverse SS18-SSX-mediated polycomb silencing of the tumor suppressor early growth response 1 in synovial sarcoma. Cancer Res. 2008;68:4303–10.

    Article  CAS  PubMed  Google Scholar 

  30. Nguyen A, Su L, Campbell B, et al. Synergism of heat shock protein 90 and histone deacetylase inhibitors in synovial sarcoma. Sarcoma. 2009;2009:794901.

    Article  PubMed  PubMed Central  Google Scholar 

  31. Su L, Sampaio AV, Jones KB, et al. Deconstruction of the SS18-SSX fusion oncoprotein complex: insights into disease etiology and therapeutics. Cancer Cell. 2012;21:333–47.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  32. Ladanyi M. Correlates of SYT-SSX Fusion Type in Synovial Sarcoma: Getting More Complex But Also More Interesting? J Clin Oncol. 2005;23:3638–9.

    Article  PubMed  Google Scholar 

  33. Guillou L, Benhattar L, Bonichon F, et al. Histologic grade, but not SYT-SSX fusion type, is an important prognostic factor in patients with synovial sarcoma: A multicenter, retrospective analysis. J Clin Oncol. 2004;22:4040–50.

    Article  PubMed  Google Scholar 

  34. Lewis JJ, Antonescu CR, Leung DH, et al. Synovial sarcoma: a multivariate analysis of prognostic factors in 112 patients with primary localized tumors of the extremity. J Clin Oncol. 2000;18:2087–94.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Brennan, M.F., Antonescu, C.R., Alektiar, K.M., Maki, R.G. (2016). Synovial Sarcoma. In: Management of Soft Tissue Sarcoma. Springer, Cham. https://doi.org/10.1007/978-3-319-41906-0_8

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-41906-0_8

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-41904-6

  • Online ISBN: 978-3-319-41906-0

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics