Diagnosis and Treatment of Soft Tissue Sarcomas

  • Miklós SzendrőiEmail author
  • Zoltan Sápi
  • Kinga Karlinger
  • Zsuzsa Pápai
Part of the European Instructional Lectures book series (EICL, volume 10)


Soft tissue tumours form a very heterogeneous group containing more than 200 clinico-pathological sub-types [15]. According to the WHO classification, all tumours originating from non-epithelial tissues, all extra-skeletal bone and cartilage-forming tumours, tumours of the peripheral nerves and neuro-ectodermal tissues are discussed here except those that develop from the reticuloendothelial system, the glia and parenchymal connective tissue. Whilst the benign soft tissue tumours are relatively common, the soft tissue sarcomas (STS) are rare, their distribution being 100:1. The annual incidence of STS is around 30/1 million inhabitants. They comprise less than 1% of all malignant tumours in adults but 15% of all paediatric malignancies [32]. The consequence of this is that the general pathologist and orthopaedic surgeon may not meet these types of tumours for years. The rarity of the STS underlines that these patients should be sent to tumour centres where enough experience is accumulated and specialized pathologists, surgeons, oncologists and radiotherapists give interdisciplinary treatment according to multi-modal protocols.


Soft Tissue Sarcoma Synovial Sarcoma Malignant Peripheral Nerve Sheath Tumour Soft Tissue Sarcoma Desmoplastic Small Round Cell Tumour 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Akerman M, Rydholm A, Persson BM (1985) Aspiration cytology of soft tissue tumours. Acta Orthop Scand 56:407–412CrossRefPubMedGoogle Scholar
  2. 2.
    Barner-Rasmussen I, Popov P, Böhling T et al (2009) Microvascular reconstruction after resection of soft tissue sarcoma of the leg. Brit J Surg 96:482–489CrossRefPubMedGoogle Scholar
  3. 3.
    Beltrami G, Rüdiger HA, Mela MM et al (2008) Limb salvage surgery in combination with brachitherapy and external beam radiation for high-grade soft tissue sarcomas. EJSO 34:811–816PubMedGoogle Scholar
  4. 4.
    Brien EW, Terek RM, Geer RJ et al (1995) Treatment of soft-tissue sarcomas of the hand. J Bone Joint Surg 77-A:564–571PubMedGoogle Scholar
  5. 5.
    Capanna R, De Biase P (2003) Excision of bone tumours. In: Duparc J (ed) Surgical techniques in orthopaedics and traumatology. Elsevier, Philadelphia, pp 55-050-B-20Google Scholar
  6. 6.
    Chansky HA (2002) Surgical management of malignant soft-tissue tumours. In: Menendez LR (ed) Orthopaedic knowledge update. Musculoskeletal tumours. AAOS, Rosemont, pp 231–241Google Scholar
  7. 7.
    Charest M, Hickeson M, Lisbona R et al (2009) PET/CT imaging in primary osseous and soft tissue sarcomas: a retrospective review of 212 cases. Eur J Nucl Med Mol Imaging. Epub. 2009 Jul 11Google Scholar
  8. 8.
    Coindre JM, Terrier Ph, Bui NB et al (1996) Prognostic factors in adult patients with locally controlled soft tissue sarcoma: a study of 546 patients from the French Federation of Cancer Centers Sarcoma Group. J Clin Oncol 14:869–877PubMedGoogle Scholar
  9. 9.
    Cormier JN, Pollock RE (2004) Soft tissue sarcomas. CA Cancer J Clin 54:94–109CrossRefPubMedGoogle Scholar
  10. 10.
    Dei Tos AP, Dal Cin P (1997) The role of cytogenetics in the classification of soft tissue tumours. Virchows Arch 431: 83–94CrossRefPubMedGoogle Scholar
  11. 11.
    Einck JP, Schwartz D (2002) Radiation therapy for malignant soft-tissue tumours. In: Menendez LR (ed) Orthopaedic knowledge update. Musculoskeletal tumours. AAOS, Rosemont, pp 289–301Google Scholar
  12. 12.
    Engellau J, Bendahl P-O, Persson A et al (2005) Improved prognostication in soft tissue sarcoma: independent information from vascular invasion, necrosis, growth pattern, and immunostaining using whole-tumour sections and tissue microarrays. Hum Pathol 36:994–1002CrossRefPubMedGoogle Scholar
  13. 13.
    Enneking WF (1983) Musculoskeletal tumour surgery. Churchill Livingstone, New York, Edinburgh, LondonGoogle Scholar
  14. 14.
    Enneking WF, Spanier SS, Goodman MA (1980) System for the surgical staging of musculoskeletal sarcoma. Clin Orthop 153:106–120PubMedGoogle Scholar
  15. 15.
    Enzinger FM, Weiss SW (1983) Soft tissue tumours. Mosby, St. Louis, Toronto, LondonGoogle Scholar
  16. 16.
    Ferrari A, Casanova M, Collini P et al (2005) Adult-type soft tissue sarcomas in pediatric-age patients: experience at the Istituto Nazionale Tumouri in Milan. J Clin Oncol 23:4021–4030CrossRefPubMedGoogle Scholar
  17. 17.
    Fletcher CDM, Rydholm A, Singer S (2002) Soft tissue tumours: epidemiology, clinical features, histopathological typing and grading. In: Fletcher CDM, Unni K, Mertens F (eds): WHO classification, tumours of soft tissue and bone. IARC Press, Lyon, pp 12–18Google Scholar
  18. 18.
    Gadgeel SM, Harlan LC, Zeruto CA et al (2009) Patterns of care in a population-based sample of soft tissue sarcoma patients in the United States. Cancer 115:2744–2754CrossRefPubMedGoogle Scholar
  19. 19.
    Gibbs CP, Peabody TD, Mundt AJ et al (1997) Oncological outcomes of operative treatment of subcutaneous soft-tissue sarcomas of the extremities. J Bone Joint Surg 79-A: 888–897PubMedGoogle Scholar
  20. 20.
    Green FL, Page DL, Fleming ID et al (2002) AJCC cancer staging manual, 6th edn. Springer, New YorkCrossRefGoogle Scholar
  21. 21.
    Gronchi A, Casali PG, Mariani L et al (2005) Status of surgical margins and prognosis in adult soft tissue sarcomas of the extremities: a series of patients treated at a single institution. J Clin Oncol 23:96–104CrossRefPubMedGoogle Scholar
  22. 22.
    Grunhagen DJ, de Wilt JHW, Graveland WJ et al (2006) Outcome and prognostic factor analysis of 217 consecutive isolated limb perfusions with tumour necrosis factor-alpha and melphalan for limb-threatening soft tissue sarcoma. Cancer 106:1776–1784CrossRefPubMedGoogle Scholar
  23. 23.
    Gustafson P, Akerman M, Alvegard TA et al (2003) Prognostic information in soft tissue sarcoma using tumour size, vascular invasion and microscopic tumour necrosis – the SIN-system. Eur J Cancer 39:1568–1576CrossRefPubMedGoogle Scholar
  24. 24.
    Ham SJ, van der Graf WTA, Pras E, Molenaar WM et al (1998) Soft tissue sarcoma of the extremities. A multimodality diagnostic and therapeutic approach. Cancer Treat Rev 24:373–391CrossRefPubMedGoogle Scholar
  25. 25.
    Harms D (1997) The pediatric tumour registry in Kiel. Acta Orthop Scand (suppl 274):65Google Scholar
  26. 26.
    Hartman JT, Bauer S (2006) Soft tissue sarcoma. Update Cancer Ther 1:385–402CrossRefGoogle Scholar
  27. 27.
    Hashimoto H, Daimaru Y, Takeshita S et al (1992) Prognostic significance of histologic parameters of soft tissue sarcomas. Cancer 70:2816–2822CrossRefPubMedGoogle Scholar
  28. 28.
    Heslin MJ, Lewis JJ, Woodruff JM et al (1997) Core needle biopsy for diagnosis of extremity soft tissue sarcoma. Ann Surg Oncol 4:425–431CrossRefPubMedGoogle Scholar
  29. 29.
    Hueman MT, Thornton K, Herman JM et al (2008) Management of extremity soft tissue sarcomas. Surg Clin N Am 88:539–557CrossRefPubMedGoogle Scholar
  30. 30.
    Iagaru A, Quon A, McDougall RI et al (2006) F-18 FDG PET/CT evaluation of osseous and soft tissue sarcomas. Clin Nucl Med 31:754–760CrossRefPubMedGoogle Scholar
  31. 31.
    Kane JM, Finley JW, Driscoll D et al (2002) The treatment and outcome of patients with soft tissue sarcomas and synchronous metastases. Sarcoma 6:69–73CrossRefPubMedGoogle Scholar
  32. 32.
    Khatri VP, Goodnight JE (2005) Extremity soft tissue sarcoma: controversal management issues. Surg Oncol 14:1–9CrossRefPubMedGoogle Scholar
  33. 33.
    Lahat G, Tuvin D, Wei C et al (2008) New perspectives for staging in soft tissue sarcoma. Ann Surg Oncol 15: 2739–2748CrossRefPubMedGoogle Scholar
  34. 34.
    Maeda M, Matsumine A, Kato H et al (2007) Soft-tissue tumours evaluated by line-scan diffusion-weighted imaging: influence of myxoid matrix on the apparent diffusion coefficient. J Magn Reson Imaging 25:1199–1204CrossRefPubMedGoogle Scholar
  35. 35.
    Mintzer DM, King JJ, Alavi A et al (2007) Utility of PET scan in predicting chemotherapeutic response in soft tissue sarcoma patients. ASCO Annual Meeting Proceedings Part I. J Clin Oncol 25(18S) (June 20 supplement):10021Google Scholar
  36. 36.
    Muhic A, Hovgaard D, Petersen MM et al (2008) Local control and survival in patientswith soft tissue sarcomas treated with limb sparing surgery in combination with interstitial brachytherapy and external radiation. Radiother Oncol 88:382–387CrossRefPubMedGoogle Scholar
  37. 37.
    Pápai Z, Bodoky G, Szántó J et al (2000) The efficacy of a combination of Etoposide, Ifosfamide and Cisplatin in the treatment of patients with soft tissue sarcoma. Cancer 89:177–200CrossRefPubMedGoogle Scholar
  38. 38.
    Pisters PWT, Leung DHY, Woodruff J et al (1996) Analysis of prognostic factors in 1041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol 14: 1679–1689PubMedGoogle Scholar
  39. 39.
    Pisters PWT, O’Sallivan B, Maki RG (2007) Evidence-based recommendations for local therapy for soft tissue sarcomas. J Clin Oncol 25:1003–1008CrossRefPubMedGoogle Scholar
  40. 40.
    Rosenberg SA, Tepper J, Glatstein E et al (1982) 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 196: 305–315CrossRefPubMedGoogle Scholar
  41. 41.
    Schwarzbach MH, Hormann Y, Hinz U et al (2005) Results of limb-sparing surgery with vascular replacement for soft tissue sarcoma in the lower extremity. J Vasc Surg 42: 88–97CrossRefPubMedGoogle Scholar
  42. 42.
    Singh HK, Kilpatrick SE, Silverman JF (2004) Fine needle aspiration biopsy of soft tissue sarcomas:utility and diagnostic challenges. Adv Anat Pathol 11:24–37CrossRefPubMedGoogle Scholar
  43. 43.
    Stefanovski PD, Bidoli E, De Paoli A et al (2002) Prognostic factors in soft tissue sarcomas: a study of 395 patients. Eur J Surg Oncol 28:153–164CrossRefPubMedGoogle Scholar
  44. 44.
    Sugiura H, Takahashi M, Katagiri H et al (2002) Additional wide resection of malignant soft tissue tumours. Clin Orthop 394:201–210CrossRefPubMedGoogle Scholar
  45. 45.
    Van Geel AN, Egermont MM, Hanssens PEJ et al (2003) Factors influencing prognosis after initial inadequate excision (IIE) for soft tissue sarcoma. Sarcoma 7:159–165CrossRefPubMedGoogle Scholar
  46. 46.
    Vraa S, Keller J, Nielsen OS et al (2001) Soft tissue sarcoma of the tigh. Surgical margin influences local recurrence but not survival in 152 patients. Acta Orthop Scand 72:72–77CrossRefPubMedGoogle Scholar
  47. 47.
    Wang CK, Li CW, Hsieh TJ et al (2004) Characterization of bone and soft-tissue tumours with in vivo 1H MR spectroscopy: initial results. Radiology 232:599–605CrossRefPubMedGoogle Scholar
  48. 48.
    Ward WG, Savage P, Boles CA et al (2001) Fine-needle aspiration biopsy of sarcomas and related tumours. Cancer Control 8:232–238PubMedGoogle Scholar
  49. 49.
    Wunder JS, Healy JH, Davis AM et al (2000) A comparison of staging systems for localized extremity soft tissue sarcoma. Cancer 88:2721–2730CrossRefPubMedGoogle Scholar
  50. 50.
    Yang JC, Chang AE, Baker AR et al (1998) Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 16:197–203PubMedGoogle Scholar
  51. 51.
    Zagara GK, Ballo MT, Pisters PW et al (2003) Surgical margins and reresection in the management of patients with soft tissue sarcoma using conservative surgery and radiation therapy. Cancer 97:2544–2553CrossRefGoogle Scholar

Copyright information

© EFORT 2010

Authors and Affiliations

  • Miklós Szendrői
    • 1
    Email author
  • Zoltan Sápi
  • Kinga Karlinger
  • Zsuzsa Pápai
  1. 1.Semmelweis University BudapestBudapestHungary

Personalised recommendations