Skip to main content
Log in

Soft-tissue Sarcomas

  • Symposium - Musculoskeletal Oncology
  • Published:
Indian Journal of Orthopaedics Aims and scope Submit manuscript

Abstract

This article discusses the epidemiology, diagnosis, and management of primary soft-tissue sarcomas (STS). These musculoskeletal tumors are a rare and heterogeneous group of malignancies, which are best managed by multidisciplinary teams in specialist sarcoma referral centers. Historically, the standard for local control of these tumors has been amputation. Evolutions in multimodality treatment have seen a shift toward preservation of the limb. Advances in limb-sparing surgery have seen the quality of life in sarcoma patients to improve drastically; however, unplanned surgical excision of STS remains a major treatment dilemma in the control of local disease.

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

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F. WHO Classification of Tumours of Soft Tissue and Bone. 4th ed. Lyon, France: IARC Press; 2013.

    Google Scholar 

  2. Siegel RL, Miller KD, Jemal A. Cancer statistics, 2015. CA Cancer J Clin 2015;65:5–29.

    Article  PubMed  Google Scholar 

  3. Hui JY. Epidemiology and etiology of sarcomas. Surg Clin North Am 2016;96:901–14.

    Article  PubMed  Google Scholar 

  4. Francis MD, Charman J, Lawrence G, Grimer R. Bone and Soft Tissue Sarcomas UK Incidence and Survival: 1996 to 2010 Version 2.0. NCIN; 2013.

  5. Zagars GK, Ballo MT, Pisters PW, Pollock RE, Patel SR, Benjamin RS, et al. Prognostic factors for patients with localized soft-tissue sarcoma treated with conservation surgery and radiation therapy: An analysis of 1225 patients. Cancer 2003;97:2530–43.

    Article  PubMed  Google Scholar 

  6. Salo JC, Lewis JJ, Woodruff JM, Leung DH, Brennan MF. Malignant fibrous histiocytoma of the extremity. Cancer 1999;85:1765–72.

    Article  CAS  PubMed  Google Scholar 

  7. Choong PF, Gustafson P, Willen H, Akerman M, Baldetrop B, Fernö M, et al. Prognosis following locally recurrent soft-tissue sarcoma. A staging system based on primary and recurrent tumour characteristics. Int J Cancer 1995;60:33–7.

    Article  CAS  PubMed  Google Scholar 

  8. Dangoor A, Seddon B, Gerrand C, Grimer R, Whelan J, Judson I, et al. UK guidelines for the management of soft tissue sarcomas. Clin Sarcoma Res 2016;6:20.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Soper JR, Brown WE, Schatz JA. Radiology of bone and soft tissue sarcomas. Cancer Forum 2010;34:126–130.

    Google Scholar 

  10. Stacy GS, Mahal RS, Peabody TD. Staging of bone tumors: A review with illustrative examples. AJR Am J Roentgenol 2006;186:967–76.

    Article  PubMed  Google Scholar 

  11. Choong P, Sim F. Tumours. Curr Orthop 2000;14:117–32.

    Article  Google Scholar 

  12. Gerrand C, Athanasou N, Brennan B, Grimer R, Judson I, Morland B, et al. UK guidelines for the management of bone sarcomas. Clin Sarcoma Res 2016;6:7.

    Article  PubMed  PubMed Central  Google Scholar 

  13. Tateishi U, Yamaguchi U, Seki K, Terauchi T, Arai Y, Kim EE, et al. Bone and soft-tissue sarcoma: Preoperative staging with fluorine 18 fluorodeoxyglucose PET/CT and conventional imaging. Radiology 2007;245:839–47.

    Article  PubMed  Google Scholar 

  14. Lin H, Ozkan E, Erkan M, Viejo-Rullan F, Mikhaeil M, Mosavi A, et al. Incremental value of FDG PET/CT in initial staging of patients (pts) with bone and soft tissue sarcoma (BSTS). J Nucl Med 2008;49 Suppl 1:149P

  15. Piperkova E, Mikhaeil M, Mousavi A, Libes R, Viejo-Rullan F, Lin H, et al. Impact of PET and CT in PET/CT studies for staging and evaluating treatment response in bone and soft tissue sarcomas. Clin Nucl Med 2009;34:146–50.

    Article  PubMed  Google Scholar 

  16. Mankin HJ, Lange TA, Spanier SS. The hazards of biopsy in patients with malignant primary bone and soft-tissue tumors. J Bone Joint Surg Am 1982;64:1121–7.

    Article  CAS  PubMed  Google Scholar 

  17. Siebenrock KA, Hertel R, Ganz R. Unexpected resection of soft-tissue sarcoma. More mutilating surgery, higher local recurrence rates, and obscure prognosis as consequences of improper surgery. Arch Orthop Trauma Surg 2000;120:65–9.

    CAS  PubMed  Google Scholar 

  18. Yao L, Nelson SD, Seeger LL, Eckardt JJ, Eilber FR. Primary musculoskeletal neoplasms: Effectiveness of core-needle biopsy. Radiology 1999;212:682–6.

    Article  CAS  PubMed  Google Scholar 

  19. Dyrop HB, Safwat A, Vedsted P, Maretty-Kongstad K, Hansen BH, Jørgensen PH, et al. Characteristics of 64 sarcoma patients referred to a sarcoma center after unplanned excision. J Surg Oncol 2016;113:235–9.

    Article  PubMed  Google Scholar 

  20. Potter BK, Adams SC, Pitcher JD Jr., Temple HT. Local recurrence of disease after unplanned excisions of high-grade soft tissue sarcomas. Clin Orthop Relat Res 2008;466:3093–100.

    Article  PubMed  PubMed Central  Google Scholar 

  21. Qureshi YA, Huddy JR, Miller JD, Strauss DC, Thomas JM, Hayes AJ, et al. Unplanned excision of soft tissue sarcoma results in increased rates of local recurrence despite full further oncological treatment. Ann Surg Oncol 2012;19:871–7.

    Article  CAS  PubMed  Google Scholar 

  22. Tedesco NS, Henshaw RM. Unplanned resection of sarcoma. J Am Acad Orthop Surg 2016;24:150–9.

    Article  PubMed  Google Scholar 

  23. Charoenlap C, Imanishi J, Tanaka T, Slavin J, Ngan SY, Chander S, et al. Outcomes of unplanned sarcoma excision: Impact of residual disease. Cancer Med 2016;5:980–8.

    Article  PubMed  PubMed Central  Google Scholar 

  24. National Collaborating Centre for Cancer, editor. Improving Outcomes for People with Sarcoma: The Manual. London: National Institute for Health and Clinical Excellence; 2006.

  25. Venkatesan M, Richards CJ, McCulloch TA, Perks AG, Raurell A, Ashford RU, et al. Inadvertent surgical resection of soft tissue sarcomas. Eur J Surg Oncol 2012;38:346–51.

    Article  CAS  PubMed  Google Scholar 

  26. Lewis JJ, Leung D, Espat J, Woodruff JM, Brennan MF. Effect of reresection in extremity soft tissue sarcoma. Ann Surg 2000;231:655–63.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Trovik CS, Bauer HC, Alvegård TA, Anderson H, Blomqvist C, Berlin O, et al. Surgical margins, local recurrence and metastasis in soft tissue sarcomas: 559 surgically-treated patients from the Scandinavian sarcoma group register. Eur J Cancer 2000;36:710–6.

    Article  CAS  PubMed  Google Scholar 

  28. Siegel HJ, Brown O, Lopez-Ben R, Siegal GP. Unplanned surgical excision of extremity soft tissue sarcomas: Patient profile and referral patterns. J Surg Orthop Adv 2009;18:93–8.

    PubMed  Google Scholar 

  29. Grimer RJ. Size matters for sarcomas! Ann R Coll Surg Engl 2006;88:519–24.

  30. Gibbs JF, Huang PR Lee RJ, McGrath B, Brooks J, McKinley B, et al. Malignant fibrous histiocytoma: An institutional review. Cancer Invest 2001;19:23–7.

    Article  CAS  PubMed  Google Scholar 

  31. Lehnhardt M, Daigeler A, Homann HH, Schwaiberger V. Goertz O, Kuhnen C, et al. MFH revisited: Outcome after surgical treatment of undifferentiated pleomorphic or not otherwise specified (NOS) sarcomas of the extremities – An analysis of 140 patients. Langenbecks Arch Surg 2009;394:313–20.

    Article  PubMed  Google Scholar 

  32. Reddy K, Holt G, Grimer R, Halpern J, Schwatrz H. editors. A Move Towards Eradicating Incompletely Excised Sarcomas – “A Tale of Two Cities”. ISOLS-MSTS Conference; 2015; Orlando, FL.; 2015.

  33. Ilaslan H, Schils J, Nageotte W, Lietman SA, Sundaram M. Clinical presentation and imaging of bone and soft-tissue sarcomas. Cleve Clin J Med 2010;77 Suppl 1:S2-7.

  34. Choong P. Principles of limb sparing surgery in bone and soft tissue sarcoma. Cancer Forum 2010;34:145–149.

    Google Scholar 

  35. Alektiar KM, Velasco J, Zelefsky MJ, Woodruff JM, Lewis JJ, Brennan MF, et al. Adjuvant radiotherapy for margin-positive high-grade soft tissue sarcoma of the extremity. Int J Radiat Oncol Biol Phys 2000;48:1051–8.

    Article  CAS  PubMed  Google Scholar 

  36. Carrle D, Bielack SS. Current strategies of chemotherapy in osteosarcoma. Int Orthop 2006;30:445–51.

    Article  PubMed  PubMed Central  Google Scholar 

  37. Ratan R, Patel SR. Chemotherapy for soft tissue sarcoma. Cancer 2016;122:2952–60.

    Article  PubMed  Google Scholar 

  38. Rosenberg SA, Tepper J, Glatstein E, Costa J, Baker A, Brennan M, et al. The treatment of soft-tissue sarcomas of the extremities: Prospective randomized evaluations of (1) limb-sparing surgery plus radiation therapy compared with amputation and (2) the role of adjuvant chemotherapy. Ann Surg 1982;196:305–15.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. Pisters PW, Harrison LB, Leung DH, Woodruff JM, Casper ES, Brennan MF, et al. Long term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. J Clin Oncol 1996;14:859–68.

    Article  CAS  PubMed  Google Scholar 

  40. Yang JC, Chang AE, Baker AR Sindelar WF, Danforth DN, Topalian SL, et al. Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 1998;16:197–203.

    Article  CAS  PubMed  Google Scholar 

  41. Harrison LB, Franzese F, Gaynor JJ, Brennan MF. Long term results of a prospective randomized trial of adjuvant brachytherapy in the management of completely resected soft tissue sarcomas of the extremity and superficial trunk. Int J Radiat Oncol Biol Phys 1993;27:259–65.

    Article  CAS  PubMed  Google Scholar 

  42. O’Connor MI, Pritchard DJ, Gunderson LL. Integration of limb-sparing surgery, brachytherapy, and external-beam irradiation in the treatment of soft-tissue sarcomas. Clin Orthop Relat Res 1993;289:73–80.

    Google Scholar 

  43. Davis AM, O’Sullivan B, Turcotte R Bell R Carton C, Chabot R, et al. Late radiation morbidity following randomization to preoperative versus postoperative radiotherapy in extremity soft tissue sarcoma. Radiother Oncol 2005;75:48–53.

    Article  PubMed  Google Scholar 

  44. O’Sullivan B, Davis AM, Turcotte R, Bell R, Carton C, Chabot R, et al. Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: A randomised trial. Lancet 2002;359:2235–41.

    Article  PubMed  Google Scholar 

  45. Ballo MT, Lee AK. Current results of brachytherapy for soft tissue sarcoma. Curr Opin Oncol 2003;15:313–8.

    Article  PubMed  Google Scholar 

  46. Holloway CL, Delaney TF, Alektiar KM, Devlin PM, O’Farrell DA, Demanes DJ, et al. American Brachytherapy Society (ABS) consensus statement for sarcoma brachytherapy. Brachytherapy 2013;12:179–90.

    Article  PubMed  Google Scholar 

  47. Blumenfeld R Sen N, Abrams R, Wang D. Advances in radiation therapy for primary and metastatic adult soft tissue sarcomas. Curr Oncol Rep 2016;18:36.

    Article  CAS  Google Scholar 

  48. O’Sullivan B, Griffin AM, Dickie CI, Sharpe MB, Chung PW, Catton CN, et al. Phase 2 study of preoperative image-guided intensity-modulated radiation therapy to reduce wound and combined modality morbidities in lower extremity soft tissue sarcoma. Cancer 2013;119:1878–84.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Peter F. M. Choong.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Vodanovich, D.A., Choong, P.F.M. Soft-tissue Sarcomas. IJOO 52, 35–44 (2018). https://doi.org/10.4103/ortho.IJOrtho_220_17

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.4103/ortho.IJOrtho_220_17

Keywords

MeSH terms

Navigation