Extremity Soft Tissue Sarcoma in Adults

  • Thomas F. DeLaney
  • David C. Harmon
  • Andrew E. Rosenberg
  • Francis J. Hornicek
Part of the Medical Radiology book series (MEDRAD)

30.8 Summary

Treatment for STS requires individual tailoring of the approach because of the wide variety of clinical situations that can arise from a tumor that involves a variety of anatomic sites with a range of histologies of variable grade and size. Nevertheless, the following suggestions can serve as useful guides. Surgery is always indicated, but the use of adjuvant therapy can vary according to the anatomic site, size, and histological grade.
  • In general, patients with superfi cial low-grade tumors that are less than 5 cm in diameter can be treated with surgical excision alone when negative margins of ≥1 cm are achieved and can expect excellent local control and survival rates approximating 90%.

  • In patients with intermediate grade lesions, surgical excision with negative margins in combination with radiotherapy has achieved excellent local control, with overall survival rates approximating 80%. For larger, deep-seated tumors, preoperative RT appears to be more effective than postoperative radiation to prevent local tumor recurrence. Acute wound healing complications are higher with preoperative RT for lower extremity lesions, but generally irreversible late complications, including grade 3–4 fi broses, are more common in those patients receiving postoperative RT.

  • In patients with high-grade STS greater than 5 cm, excellent local control can be achieved with surgery and radiotherapy, but at least 50% of these patients will develop metastatic disease. In this setting, the use of neoadjuvant chemotherapy may benefi t some and should be considered in the context of a clinical trial, to be followed by defi nitive surgery combined with either pre- or postoperative radiotherapy or BRT.

  • BRT can provide excellent local control and functional results in appropriately selected patients.


Clin Oncol Soft Tissue Sarcoma Radiat Oncol Biol Phys Synovial Sarcoma Soft Tissue Sarcoma 
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.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Abbas JS, Holyoke ED, Moore R et al (1981) The surgical treatment and outcome of soft-tissue sarcoma. Arch Surg 116:765–769PubMedGoogle Scholar
  2. Alektiar KM, Zelefsky MJ, Brennan MF (2000) Morbidity of adjuvant brachytherapy in soft tissue sarcoma of the extremity and superficial trunk. Int J Radiat Oncol Biol Phys 47:1273–1279PubMedCrossRefGoogle Scholar
  3. Alvegard TA, Sigurdsson H, Mouridsen H et al (1989) Adjuvant chemotherapy with doxorubicin in high-grade soft tissue sarcoma: a randomized trial of the Scandinavian Sarcoma Group. J Clin Oncol 7:1504–1513PubMedGoogle Scholar
  4. Aman P, Ron D, Fioretos T et al (1992) Rearrangement of the transcription factor gene CHOP in myxoid liposarcomas with t(12;16)(q13;p11). Genes Chromosomes Cancer 5:278PubMedGoogle Scholar
  5. American Joint Committee on the Staging of Cancer Staging System for Soft Tissue Sarcomas (2002) AJCC cancer staging manual. Springer, Belrin Heidelberg New YorkGoogle Scholar
  6. Angervall L, Kindblom LG (1993) Principles for pathologicanatomic diagnosis and classification of soft-tissue sarcomas. Clin Orthop 289:9–18PubMedGoogle Scholar
  7. Antman KH (1997) Adjuvant therapy of sarcomas of soft tissue. Semin Oncol 24:556–560PubMedGoogle Scholar
  8. Arbeit JM, Hilaris B, Brennan MF (1987) Wound complications in the multimodality treatment of extremity and superficial truncal sarcomas. J Clin Oncol 5:480–488PubMedGoogle Scholar
  9. Austin-Seymour M, Munzenrider J, Goitein M et al (1989) Fractionated proton radiation therapy of chordoma and low grade chondrosarcoma of the base of skull. J Neurosurg 70:13–17PubMedCrossRefGoogle Scholar
  10. Baldini EH, Goldberg J, Jenner C et al (1999) Long-term outcomes after function-sparing surgery without radiotherapy for soft tissue sarcoma of the extremities and trunk. J Clin Oncol 17:3252–3259PubMedGoogle Scholar
  11. Bell RS, O’Sullivan B, Liu FF et al (1989) The surgical margin in soft tissue sarcoma. J Bone Joint Surg 71:370–375PubMedGoogle Scholar
  12. Billingsley KG, Lewis JJ, Leung DHY et al (1999) Multifactorial analysis of the survival of patients with distant metastasis arising from primary extremity sarcoma. Cancer 85:389–395PubMedCrossRefGoogle Scholar
  13. Bland KI, McCoy DM, Kinnard RE et al (1987) Application of magnetic resonance imaging and computerized tomography as an adjunct to the surgical management of soft tissue sarcomas. Ann Surg 205:473–481PubMedGoogle Scholar
  14. Blay J-Y, van Glabbeke M, Verweij J et al (2003) Advanced soft-tissue sarcoma: a disease that is potentially curable for a subset of patients treated with chemotherapy. Eur J Cancer 39:64–69PubMedCrossRefGoogle Scholar
  15. Blum RH, Edmonson J, Ryan L et al (1993) Efficacy of ifosfamide in combination with doxorubicin for the treatment of metastatic soft-tissue sarcoma. The Eastern Cooperative Oncology Group. Cancer Chemother Pharmacol 31[Suppl 2]:S238–S240PubMedGoogle Scholar
  16. Boulad F, Kernan NA, LaQuaglia MP et al (1998) High-dose induction chemoradiotherapy followed by autologous bone marrow transplantation as consolidation therapy in rhabdomyosarcoma, extraosseous Ewing’s sarcoma, and undifferentiated sarcoma. J Clin Oncol 16:1697PubMedGoogle Scholar
  17. Bramwell VH (1997) The role of chemotherapy in the management of non-metastatic operable extremity osteosarcoma. Semin Oncol 24:561PubMedGoogle Scholar
  18. Bramwell VH, Mouridsen HT, Santoro A et al (1993) Cyclophosphamide versus ifosfamide: a randomized Phase 11 trial in adult soft-tissue sarcomas. The European Organization for Research and Treatment of Cancer [EORTC], Soft Tissue and Bone Sarcoma Group. Cancer Chemother Pharmacol 31[Suppl 2]:S180–S184PubMedGoogle Scholar
  19. Brant TA, Parsons JT, Marcus RB et al (1990) Preoperative irradiation for soft tissue sarcomas of the trunk and extremities in adults. Int J Radiat Oncol Biol Phys 19:899–906PubMedGoogle Scholar
  20. Brodowicz T, Schwameis E, Widder J et al (2000) Intensified adjuvant IFADIC chemotherapy for adult soft tissue sarcoma. A prospective randomized feasibility trial. Sarcoma 4:151PubMedGoogle Scholar
  21. Brown R, Marshall CJ, Pennie SG et al (1984) Mechanism of activation of an N-ras gene in the human fibrosarcoma cell line HT1080. EMBO J 3:1321–1326PubMedGoogle Scholar
  22. Bujko K, Suit HD, Springfield S et al (1992) Wound healing after surgery and preoperative radiation for sarcoma of soft tissues. Surg Gynecol Obstet 176:124–134Google Scholar
  23. Catton C, O’Sullivan B, Bell R et al (1996a) Chordoma: long-term follow-up after radical photon irradiation. Radiother Oncol 41:67–72PubMedGoogle Scholar
  24. Catton C, Davis A, Bell R et al (1996b) Soft tissue sarcoma of the extremity. Limb salvage after failure of combined conservative therapy. Radiother Oncol 41:209–214PubMedCrossRefGoogle Scholar
  25. Chan MF, Chui CS, Shupak K et al (2001) The treatment of large extraskeletal chondrosarcoma of the leg: comparison of IMRT and conformal radiotherapy techniques. J Appl Clin Med Phys 2:3–8PubMedCrossRefGoogle Scholar
  26. Christman KL, Casper ES, Schwartz JK et al (1993) High-intensity scheduling of ifosfamide in adult patients with soft-tissue sarcoma. Proc Ann Meet Am Soc Clin Oncol 12:A1642Google Scholar
  27. Clark J, Rocques PJ, Crew AJ et al (1994) Identification of novel genes, SYT and SSX, involved in the t(X;18)(p11.2;q11.2) translocation found in human synovial sarcoma. Nat Genet 7:502PubMedCrossRefGoogle Scholar
  28. Clark MA, FIsher C, Judson I, Thomas JM (2005) Soft-tissue sarcomas in adults. N Engl J Med 22:591–604Google Scholar
  29. Cleator SJ, Cottril C, Harmer C et al (2001) Pattern of local recurrence after conservative surgery and radiotherapy for soft tissue sarcoma. Sarcoma 5:83–88PubMedGoogle Scholar
  30. Coindre JM, Terrier P, Bui B 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
  31. Cormier JN, Huang X, Xing Y et al (2004) Cohort analysis of patients with localized, high-risk, extremity soft tissue sarcoma treated at two cancer centers: chemotherapy-associated outcomes. J Clin Oncol 22:4567PubMedCrossRefGoogle Scholar
  32. Costa J, Wesley RA, Glatstein E et al (1984) The grading of soft tissue sarcomas. Results of a clinicohistopathologic correlation in a series of 163 cases. Cancer 53:530–541Google Scholar
  33. Crist WM, Anderson JR, Meza JL et al (2001) Intergroup rhabdomyosarcoma study-IV: results for patients with nonmetastatic disease. J Clin Oncol 19:3091–3102PubMedGoogle Scholar
  34. Cruz AB, Thames EA Jr, Aust JB et al (1979) Combination chemotherapy for soft tissue sarcomas: a phase III study. J Surg Oncol 11:313–323PubMedGoogle Scholar
  35. Davis AM, Devlin M, Griffin AM et al (1999) Functional outcome in amputation versus limb sparing of patients with lower extremity sarcoma: a matched case-control study. Arch Phys Med Rehabil 80:615–618PubMedCrossRefGoogle Scholar
  36. Davis AM, O’Sullivan B, Bell RS et al (2002) Function and health status outcomes in a randomized trial comparing preoperative and postoperative radiotherapy in extremity soft tissue sarcoma. J Clin Oncol 20:4472–4477PubMedCrossRefGoogle Scholar
  37. DeChiara A, T’Ang A, Triche TJ (1993) Expression of the retinoblastoma susceptibility gene in childhood rhabdomyosarcomas. J Natl Cancer Inst 85:152–157Google Scholar
  38. DeLaney TF, Rosenberg AE, Harmon DC et al (2003a) Soft tissue sarcomas. In: Price PM, Sikora K (eds) Treatment of cancer. Arnold, London, pp 869–907Google Scholar
  39. DeLaney TF, Spiro IJ, Suit HD et al (2003b) Neoadjuvant chemotherapy and radiotherapy for large extremity soft-tissue sarcomas. Int J Radiat Oncol Biol Phys 56:1117–1127PubMedCrossRefGoogle Scholar
  40. Delattre O, Zucman J, Merlot T et al (1994) The Ewing family of tumors-a subgroup of small round cell tumors defined by specific chimeric transcripts. N Engl J Med 331:294–299PubMedCrossRefGoogle Scholar
  41. Dewhirst MW, Boatman HD, Leopold KA et al (1990) Soft-tissue sarcomas: MR imaging and MR spectrosopy for prognosis and therapy monitoring. Radiology 174:847–853PubMedGoogle Scholar
  42. Dias P, Dealing M, Houghton P (1994) The molecular basis of skeletal muscle differentiation. Semin Diagn Pathol 11:3–14PubMedGoogle Scholar
  43. Dumontet C, Biron P, Bouffet E et al (1992) High dose chemotherapy with ABMT in soft tissue sarcomas: a report of 22 cases. Bone Marrow Transplant 10:405PubMedGoogle Scholar
  44. Edmonson JH, Ryan LM, Blum RH et al (1993) Randomized comparison of doxorubicin alone versus ifosfamide plus doxorubicin or mitomycin, doxorubicin, and Cisplatin against advanced soft tissue sarcomas. J Clin Oncol 11:1269–1275PubMedGoogle Scholar
  45. Eilber FR, Eckardt J (1997) Surgical management of soft tissue sarcomas. Semin Oncol 24:526–533PubMedGoogle Scholar
  46. Eilber F, Eckardt J, Rosen G et al (1995) Preoperative therapy for soft tissue sarcoma. Hematol Oncol Clin North Am 9:817–823PubMedGoogle Scholar
  47. Eilber FC, Rosen G, Eckardt J et al (2001) Treatment-induced pathologic necrosis: a predictor of local recurrence and survival in patients receiving neoadjuvant therapy for high-grade extremity soft tissue sarcomas. J Clin Oncol 19:3203–3209PubMedGoogle Scholar
  48. Eilber FC, Rosen G, Nelson SD et al (2003) High-grade extremity soft tissue sarcomas: factors predictive of local recurrence and its effect on morbidity and mortality. Ann Surg 237:218–226PubMedCrossRefGoogle Scholar
  49. Elias A, Ryan L, Sulkes A et al (1989) Response to mesna, doxorubicin, ifosfamide and dacarbazine in 108 patients with metastatic or unresectable sarcoma and no prior chemotherapy. J Clin Oncol 7:1208–1216PubMedGoogle Scholar
  50. Elias AD, Antman KH (1986) Doxorubicin, ifosfamide, and dacarbazine (AID) with mesna uroprotection for advanced untreated sarcoma: a phase I study. Cancer Treatment Rep 70:827–833Google Scholar
  51. Engellau J (2004) Prognostic factors in soft tissue sarcoma: tissue microarray for immunostaining, the importance of whole-tumor sections and time-dependence. Acta Orthop Scand 75[Suppl 314]:1–52CrossRefGoogle Scholar
  52. Fata F, O’Reilly E, Ilson D et al (1999) Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face. Cancer 86:2034–2037PubMedCrossRefGoogle Scholar
  53. Fein DA, Lee WR, Lanciano RM et al (1995) Management of extremity soft tissue sarcomas with limb-sparing surgery and postoperative irradiation: do total dose, overall treatment time, and the surgery-radiotherapy interval impact on local control? Int J Radiat Oncol Biol Phys 32:969–976PubMedCrossRefGoogle Scholar
  54. Ferrari A, Gronchi A, Casanova M et al (2004) Synovial sarcoma: a retrospective analysis of 271 patients of all ages treated at a single institution. Cancer 101:627–634PubMedCrossRefGoogle Scholar
  55. Fong Y, Coal DG, Woodruff JM et al (1993) Lymph node metastasis from soft tissue sarcoma in adults. Analysis of data from a prospective data base of 1772 sarcoma patients. Ann Surg 217:72–77Google Scholar
  56. Frustaci S, Gherlinzoni F, DePaoli A et al (2001) Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian Cooperative Trial. J Clin Oncol 19:1238–1247PubMedGoogle Scholar
  57. Gadd MA, Casper ES, Woodruff J et al (1993) Development and treatment of pulmonary metastases in adult patients with extremity soft tissue sarcoma. Ann Surg 218:705–712PubMedGoogle Scholar
  58. Gerrand CH, Wunder JS, Kandel A et al (2004) The influence of anatomic location on functional outcome in lower-extremity soft-tissue sarcoma. Ann Surg Oncol 11:476–482. Epub 12.4.2004PubMedCrossRefGoogle Scholar
  59. Goffman T, Tochner Z, Glatstein E et al (1991) Primary treatment of large and massive adult sarcomas with iododeoxyuridine and aggressive hyperfractionated irradiation. Cancer 67:572–576PubMedCrossRefGoogle Scholar
  60. Gottlieb JA, Baker LH, Quagliana JM et al (1972) Chemotherapy of sarcomas with a combination of adriamycin and dimethyl triazeno imidazole carboxamide. Cancer 30:1632–1638PubMedCrossRefGoogle Scholar
  61. Gottlieb JA, Benjamin RS, Baker LH et al (1976) Role of DTIC (NSC45388) in the chemotherapy of sarcomas. Cancer Treat Rep 60:199–203PubMedGoogle Scholar
  62. Grabois M, Frappaz D, Bouffet E et al (1994) High-dose VP-16 cisplatinum in soft tissue sarcomja of children. Cancer Chemother Pharmacol 33:355–357PubMedCrossRefGoogle Scholar
  63. Grobmyer SR, Maki RG, Demetri GD et al (2004) Neo-adjuvant chemotherapy for primary high-grade extremity soft tissue sarcoma. Ann Oncol 15:1667–1672PubMedCrossRefGoogle Scholar
  64. 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 23:96–104PubMedCrossRefGoogle Scholar
  65. Gustafson P, Herrlin K, Biling L et al (1992) Necrosis observed on CT enhancement is of prognostic value in soft tissue sarcoma. Acta Radiol 33:474–476PubMedCrossRefGoogle Scholar
  66. Harrison LB, Franzese F, Gaynor JJ et al (1993) 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 27:259–265PubMedGoogle Scholar
  67. Hensley ML, Maki RG, Venkatraman E et al (2002) Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: results of a phase II trial. J Clin Oncol 20:2824–2831PubMedCrossRefGoogle Scholar
  68. Hong L, Alektiar KM, Hunt M et al (2004) Intensity-modulated radiotherapy for soft tissue sarcoma of the thigh. Int J Radiat Oncol Biol Phys 59:752–759PubMedCrossRefGoogle Scholar
  69. Jemal A, Murray T, Ward E et al (2005) Cancer statistics, 2005. CA Cancer J Clin 55:10–30PubMedCrossRefGoogle Scholar
  70. Johnstone PAS, Wexler LH, Venzon DJ et al (1994) Sarcomas of the hand and foot: analysis of local control and functional result with combined modality therapy in extremity preservation. Int J Radiat Oncol Biol Phys 29:735–745PubMedGoogle Scholar
  71. Judson I (2004) Systemic therapy of soft tissue sarcoma: an improvement in outcome. Ann Oncol 15[Suppl 4]:iv193–iv196PubMedGoogle Scholar
  72. Karakousis CP, Holtermann OA, Holyoke ED (1979) Cisdichlorodiammineplatinum (II) in metastatic soft tissue sarcomas. Cancer Treat Rep 63:2071–2072PubMedGoogle Scholar
  73. Karakousis CP, Emrich LJ, Rao U et al (1986) Feasibility of limb salvage and survival in soft tissue sarcomas. Cancer 57:484–491PubMedCrossRefGoogle Scholar
  74. Karakousis CP, Proimakis C, Rao U et al (1996) Local recurrence and survival in soft-tissue sarcomas. Ann Surg Oncol 3:255–260PubMedCrossRefGoogle Scholar
  75. Karakousis CP, de Young C, Driscoll DL et al (1998) Soft tissue sarcomas of the hand and foot: management and survival. Ann Surg Oncol 5:238–240PubMedCrossRefGoogle Scholar
  76. Karakousis CP, Driscoll DL (1999) Treatment and local control of primary extremity soft tissue sarcomas. J Surg Oncol 71:155–161PubMedCrossRefGoogle Scholar
  77. Kepka L, DeLaney TF, Goldberg SI et al (2005) Results of radiation therapy for unresected soft tissue sarcomas. Int J Radiat Oncol Biol Phys 63:852–859PubMedCrossRefGoogle Scholar
  78. Koutcher JA, Ballon D, Graham M et al (1990) 31P NMR spectra of extremity sarcomas: diversity of metabolic profiles and changes in response to chemotherapy. Magn Reson Imaging Med 16:19–34Google Scholar
  79. Kransdorf MJ, Jelinek JS, Moser RP et al (1993) Imaging of soft tissue tumors. Radiol Clin North Amer 31:359–371Google Scholar
  80. Kraybill WG, Spiro I, Harris J et al (2001) Radiation Therapy Oncology Group (RTOG) 95-14: a phase II study of neoadjuvant chemotherapy (CT) and radiation therapy (RT) in high risk (HR), high grade, soft tissue sarcomas (STS) of the extremities and body wall: a preliminary report. Proc ASCO 20:348aGoogle Scholar
  81. Kretzler A, Molls M, Gradinger R et al (2004) Intraoperative radiotherapy of soft tissue sarcoma of the extremity. Perioperative fractionated high-dose rate brachytherapy in the treatment of soft tissue sarcomas. Strahlenther Onkol 180:365–370PubMedCrossRefGoogle Scholar
  82. Kulander BG, Polissar L, Yang CY et al (1989) Grading of soft tissue sarcomas: necrosis as a determinate of survival. Modern Pathol 2:205–208Google Scholar
  83. Lack EE, Steinberg SM, White DE et al (1989) Extremity soft tissue sarcomas: analysis of prognostic variables in 300 cases and evaluation of tumor necrosis as a factor in stratifying higher grade sarcomas. J Surg Oncol 263:73Google Scholar
  84. Lang H, Nussbaum KT, Kaudel P et al (2000) Hepatic metastases from leiomyosarcoma: a single-center experience with 34 liver resections during a 15-year period. Ann Surg 231:500PubMedCrossRefGoogle Scholar
  85. Lawrence W Jr, Donegan WL, Natarajan N et al (1987) Adult soft tissue sarcomas. A pattern of care survey of the American College of Surgeons. Ann Surg 205:349–359PubMedGoogle Scholar
  86. Le Cesne A, Judson I, Crowther D et al (2000) Randomized phase III study comparing conventional-dose doxorubicin plus ifosfamide versus high-dose doxorubicin plus ifosfamide plus recombinant human granulocyte-macrophage colony-stimulating factor in advanced soft tissue sarcomas: a trial of the European Organization for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group. J Clin Oncol 18:2676–2684PubMedGoogle Scholar
  87. Lee N, Chuang C, Quivey JM et al (2002) Skin toxicity due to intensity-modulated radiotherapy for head-and-neck carcinoma. Int J Radiat Oncol Biol Phys 53:630–637PubMedCrossRefGoogle Scholar
  88. LeVay J, O’Sullivan B, Catton C et al (1993) Outcome and prognostic factors in soft tissue sarcoma in the adult. Int J Radiat Oncol Biol Phys 27:1091PubMedGoogle Scholar
  89. Lindberg RD, Martin R. Romsdahl M et al (1981) Conservative surgery and postoperative radiotherapy in 300 adults with soft-tissue sarcomas. Cancer 47:2391–2397PubMedCrossRefGoogle Scholar
  90. Maki RG (2004) Role of chemotherapy in patients with soft tissue sarcomas. Exp Rev Anticancer Ther 4:229–236CrossRefGoogle Scholar
  91. Markhede G, Angervall L, Stener B et al (1981) A multivariate analysis of the prognosis after surgical treatment of malignant soft tissue tumors. Cancer 40:1721–1733Google Scholar
  92. Mazeron JJ, Suit HD (1987) Lymph nodes as sites of metastasis from sarcomas of soft tissue. Cancer 60:1800–1808PubMedCrossRefGoogle Scholar
  93. Merimsky O, Meller I, Issakov J et al (1999) Adriamycin-ifosfamide induction chemotherapy for extremity soft tissue sarcoma: comparison of two non-randomized protocols. Oncol Rep 6:913–920PubMedGoogle Scholar
  94. Mundt AJ, Awan A, Sibley S et al (1995) Conservative surgery and adjuvant radiation therapy in the management of adult soft tissue sarcoma of the extremities: clinical and radiobiological results. Int J Radiat Oncol Biol Phys 32:977–985PubMedCrossRefGoogle Scholar
  95. Myhre-Jensen, Kaae S, Madsen EH et al (1975) Histopathological grading in soft tissue tumors. Acta Pathol Microbiol Immunol Scand [A] 91:145–150Google Scholar
  96. Nag S, Shasha D, Janjan N et al (2001) The American Brachytherapy Society recommendations for brachytherapy of soft tissue sarcomas. Int J Radiat Oncol Biol Phys 49:1033–1043PubMedCrossRefGoogle Scholar
  97. Negendank WG, Crowley MG, Ryan JR et al (1989) Bone and soft tissue lesions: diagnosis with combined H-1 MR imaging and P-31 spectroscopy. Radiology 173:181–187PubMedGoogle Scholar
  98. Niewald M, Berberich W, Schnabel K et al (1990) A simple method for positioning and fixing the extremities during the radiotherapy of soft-tissue sarcomas. Strahlenther Onkol 166:295–296PubMedGoogle Scholar
  99. Nieweg OE, Pruim J, van Ginkel J et al (1996) Fluorine-18-Flurodeoxyglucose PET imaging of soft-tissue sarcoma. J Nucl Med 37:257–261PubMedGoogle Scholar
  100. Noria S, Davis A, Krandel R et al (1996) Residual disease following unplanned excision of soft-tissue sarcoma of an extremity. J Bone Joint Surg Am 78:650–655PubMedGoogle Scholar
  101. O’Bryan RM, Baker LH, Gottleib JE et al (1977) Dose response evaluation of adriamycin in human neoplasia. Cancer 39:1940–1948PubMedCrossRefGoogle Scholar
  102. O’Keefe F, Lorigan JG, Wallace S et al (1990) Radiological features of extraskeletal Ewing sarcoma. Br J Radiol 63:456–460CrossRefGoogle Scholar
  103. O’Sullivan B, Davis A, Bell R et al (1999) Phase III randomized trial of pre-operative versus post-operative radiotherapy in the curative management of extremity soft tissue sarcoma. A Canadian Sarcoma Group and NCI Canada Clinical Trials Group study. Proc ASCO 18:2066AGoogle Scholar
  104. O’Sullivan B, Davis AM, Turcotte R et al (2002) Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomized trial. Lancet 359:2235–2241PubMedCrossRefGoogle Scholar
  105. O’Sullivan B, Davis AM, Turcotte R et al (2004) Five-year results of a randomized phase III trial of pre-operative vs. post-operative radiotherapy in extremity soft tissue sarcoma. Proc Am Soc Clin Oncol 23:815Google Scholar
  106. Panagopoulos I, Hoglund M, Mertens F et al (1996) Fusion of EWS and CHOP genes in myxoid liposarcoma. Oncogene 12:489–494PubMedGoogle Scholar
  107. Pao WJ, Pilepich MV (1990) Postoperative radiotherapy in the treatment of extremity soft tissue sarcomas. Int J Radiat Oncol Biol Phys 19:907–911PubMedGoogle Scholar
  108. Parham DM, Shapiro DN, Downing R et al (1994) Solid alveolar rhabdomyosarcomas with the t(2;13). Report of two cases with diagnostic implications. Am J Surg Pathol 18:474–478PubMedCrossRefGoogle Scholar
  109. Pearlstone DB, Janjan NA, Feig W et al (1999a) Re-resection with brachytherapy for locally recurrent soft tissue sarcoma arising in a previously radiated field (see comments). Cancer J Sci Am 5:26–33PubMedGoogle Scholar
  110. Pearlstone DB, Pisters PW, Bold J et al (1999b) Patterns of recurrence in extremity liposarcoma: Implications for staging and follow-up. Cancer 85:85–92PubMedCrossRefGoogle Scholar
  111. Petera J, Neumanova R, Odrazka K et al (2004) Perioperative fractionated high-dose rate brachytherapy in the treatment of soft tissue sarcomas. Neoplasma 51:59–63PubMedGoogle Scholar
  112. Petrioli R, Coratti A, Correale P et al (2002) Adjuvant epirubicin with or without Ifosfamide for adult soft-tissue sarcoma. Am J Clin Oncol 25:468–473PubMedCrossRefGoogle Scholar
  113. Peuchot M, Libshitz HI (1987) Pulmonary metastatic disease: radiologic-surgical correlation. Radiology 164:719–722PubMedGoogle Scholar
  114. Pickering DG, Stewart JS, Rampling R et al (1987) Fast neutron therapy for soft tissue sarcoma. Int J Radiat Oncol Biol Phys 13:1489–1495PubMedGoogle Scholar
  115. Pinedo HM, Verweij J (1986) The treatment of soft tissue sarcomas with a focus on chemotherapy: a review. Radiother Oncol 6:193–205Google Scholar
  116. Pisters PW, Harrison LB, Leung DH et al (1996a) Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. J Clin Oncol 14:859–868PubMedGoogle Scholar
  117. Pisters PW, Leung DH, Woodruff J et al (1996b) Analysis of prognostic factors in 1041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol 14:1679–1689PubMedGoogle Scholar
  118. Pogrebniak HW, Roth JA, Steinberg M et al (1991) Reoperative pulmonary resection in patients with metastatic soft tissue sarcoma. Ann Thorac Surg 52:197–203PubMedCrossRefGoogle Scholar
  119. Potter DA, Glenn J, Kinsella T et al (1985) Patterns of recurrence in patients with high-grade soft tissue sarcomas. J Clin Oncol 3:353–366PubMedGoogle Scholar
  120. Prosnitz LR, Maguire P, Anderson JM et al (1999) The treatment of high-grade soft tissue sarcomas with preoperative thermoradiotherapy. Int J Radiat Oncol Biol Phys 45:941–949PubMedCrossRefGoogle Scholar
  121. Redmond OM, Bell E, Stack JP et al (1992) Tissue characterization and assessment of preoperative chemotherapeutic response in musculoskeletal tumors by in vivo 31p magnetic resonance spectroscopy. Magn Reson Med 27:226–237PubMedGoogle Scholar
  122. Rhomberg W, Hassenstein EO, Gefeller D et al (1996) Radiotherapy vs. radiotherapy and razoxane in the treatment of soft tissue sarcomas: final results of a randomized study. Int J Radiat Oncol Biol Phys 36:1077–1084PubMedCrossRefGoogle Scholar
  123. Robinson M, Barr L, Fisher C et al (1990) Treatment of extremity soft tissue sarcomas with surgery and radiotherapy. Radiother Oncol 18:221–233PubMedCrossRefGoogle Scholar
  124. Robinson MH, Ball AB, Schofield J et al (1992) Preoperative radiotherapy for initially inoperable extremity soft tissue sarcomas. Clin Oncol (R Coll Radiol) 4:36–43Google Scholar
  125. Robinson MH, Spruce L, Eeles R et al (1991) Limb function following conservation treatment of adult soft tissue sarcoma. Eur J Cancer 27:1567–1574PubMedCrossRefGoogle Scholar
  126. Rosen G, Caparros B, Nirenberg A et al (1981) Ewing’s sarcoma: ten-year experience with adjuvant chemotherapy. Cancer 47:2204–2213PubMedCrossRefGoogle Scholar
  127. 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 196:305–315PubMedGoogle Scholar
  128. Ruka W, Taghian A, Gioioso D et al (1996) Comparison between the in vitro intrinsic radiation sensitivity of human soft tissue sarcoma and breast cancer cell lines. J Surg Oncol 61:290–294PubMedCrossRefGoogle Scholar
  129. Russell WO, Cohen J, Enzinger F et al (1977) A clinical and pathological staging system for soft tissue sarcomas. Cancer 40:1562–1570PubMedCrossRefGoogle Scholar
  130. Rydholm A, Berg NO, Gullberg B et al (1984) Epidemiology of soft tissue sarcoma in the locomotor system. Acta Pathol Microbiol Immunol Scand [A] 92:363–374Google Scholar
  131. Rydholm A, Gustafson P, Rooser B et al (1991) Limb-sparing surgery without radiotherapy based on anatomic location of soft tissue sarcoma. J Clin Oncol 9:1757–1765PubMedGoogle Scholar
  132. Sadoski C, Suit HD, Rosenberg A et al (1993) Preoperative radiation, surgical margins, and local control of extremity sarcomas of soft tissues. J Surg Oncol 52:223–230PubMedGoogle Scholar
  133. Sanchez RB, Quinn SF, Walling A et al (1990) Musculoskeletal neoplasms after intraarterial chemotherapy: correlation of MR images with pathologic specimens. Radiology 174:237–240PubMedGoogle Scholar
  134. Santoro A, Tursz T, Mouridsen H et al (1995) Doxorubicin versus CYVADIC versus doxorubicin plus ifosfamide in first-line treatment of advanced soft tissue sarcomas: a randomized study of the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group. J Clin Oncol 13:1537–1545PubMedGoogle Scholar
  135. Sarcoma Meta-Analysis Collaboration (1997) Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Lancet 350:1647–1654CrossRefGoogle Scholar
  136. Schmitt G, Pape H, Zamboglou M et al (1990) Long term results of neutron-and neutron-boost irradiation of soft tissue sarcomas. Strahlenther Onkol 166:61–62PubMedGoogle Scholar
  137. Schoenfeld DA, Rosenbaum C, Horton J et al (1982) A comparison of adriamycin versus vincristine and adriamycin, and cyclophosphamide versus vincristine, actinomycin-D, and cyclophosphamide for advanced sarcoma. Cancer 50:2757–2762PubMedCrossRefGoogle Scholar
  138. Schray MF, Gunderson LL, Sim FH et al (1990) Soft tissue sarcomas. Integration of brachytherapy, resection, and external irradiation. Cancer 66:451–456PubMedCrossRefGoogle Scholar
  139. Schuetze SM, Rubin BP, Vernon C et al (2005) Use of positron emission tomoography in localized extremity soft tissue sarcoma treated with neoadjuvant chemotherapy. Cancer 103:339–348PubMedCrossRefGoogle Scholar
  140. Schutte J, Mouridsen HT, Steward W et al (1993) Ifosfamide plus doxorubicin in previously untreated patients with advanced soft-tissue sarcoma. Cancer Chemother Pharmacol 31[Suppl 2]:S204–S209PubMedGoogle Scholar
  141. Schwella N, Rick O, Meyer O et al (1998) Mobilization of peripheral blood progenitor cells by disease-specific chemotherapy in patients with soft tissue sarcoma. Bone Marrow Transplant 21:863–868PubMedCrossRefGoogle Scholar
  142. Scurr M, Juson I (2005) Neoadjuvant and adjuvant therapy for extremity soft tissue sarcoma. Hematol Oncol Clin North Am 19:489–500PubMedCrossRefGoogle Scholar
  143. Simon MA, Enneking WF (1976) The management of softtissue sarcomas of the extremities. J Bone Joint Surg Am 58:317–327PubMedGoogle Scholar
  144. Spillane AJ, Fisher C, Thomas JM et al (1999) Myxoid liposarcoma-the frequency and the natural history of nonpulmonary soft tissue metastases. Ann Surg Oncol 6:389–394PubMedCrossRefGoogle Scholar
  145. Steingraber M, Lessel A, Jahn U et al (1996) Fast neutron therapy in treatment of soft tissue sarcoma-the Berlin-Buch study. Bull Cancer Radiother 83[Suppl]:122s–124sPubMedGoogle Scholar
  146. Steward WP, Verweij J, Somers R et al (1993) Granulocyte-macrophage colony-stimulating factor allows safe escalation of dose-intensity of chemotherapy in metastatic adult soft tissue sarcomas: a study of the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group. J Clin Oncol 11:15–21PubMedGoogle Scholar
  147. Stinson SF, DeLaney TF, Greenberg J et al (1991) Acute and long term effects on limb function of combined modality limb sparing therapy for extremity soft tissue sarcomas. Int J Radiat Oncol Biol Phys 21:1493–1499PubMedGoogle Scholar
  148. Subramanian S, Wiltshaw E (1978) Chemotherapy of sarcoma. Lancet 1:683–686PubMedCrossRefGoogle Scholar
  149. Sugarbaker PH, Barofsky I, Rosenberg SA et al (1982) Quality of life assessment of patients in extremity sarcoma clinical trials. Surgery 91:17–23PubMedGoogle Scholar
  150. Suit HD, Russell WO, Martin RG et al (1975) Sarcoma of soft tissue: clinical and histopathologic parameters and response to treatment. Cancer 35:1478–1483PubMedCrossRefGoogle Scholar
  151. Suit HD, Mankin HJ, Wood WC et al (1988) Treatment of the patient with stage M0 sarcoma of soft tissue. J Clin Oncol 6:854–862PubMedGoogle Scholar
  152. Talbert ML, Zagars GK, Sherman NE et al (1990) Conservative surgery and radiation therapy for soft tissue sarcoma of the wrist, hand, ankle, and foot. Cancer 66:2482–2491PubMedCrossRefGoogle Scholar
  153. Tanabe KK, Pollock RE, Ellis LM et al (1994) Influence of surgical margins on outcome in patients with preoperatively irradiated extremity soft tissue sarcomas. Cancer 73:1652–1659PubMedCrossRefGoogle Scholar
  154. Tepper JE, Suit HD (1985) Radiation therapy alone for sarcoma of soft tissue. Cancer 56:475–479PubMedCrossRefGoogle Scholar
  155. Tepper J, Rosenberg SA, Glatstein E et al (1982) Radiation therapy technique in soft tissue sarcomas of the extremity-policies of treatment at the National Cancer Institute. Int J Radiat Oncol Biol Phys 8:263–273PubMedGoogle Scholar
  156. Todoroki T, Suit HD (1985) Therapeutic advantage in preoperative single-dose radiation combined with conservative and radical surgery in different-size murine fibrosarcomas. J Surg Oncol 29:207–215PubMedGoogle Scholar
  157. Torosian MH, Friedrich C, Godbold J et al (1988) Soft-tissue sarcoma: initial characteristics and prognostic factors in patients with and without metastatic disease. Semin Surg Oncol 4:13–19PubMedGoogle Scholar
  158. Trojani M (1984) Staging system for soft tissue and bone. Int J Cancer 33:37–42PubMedGoogle Scholar
  159. Trovik CS, Bauer HC, Alvegard A et al (2000) Surgical margins, local recurrence and metastasis in soft tissue sarcomas: 559 surgically-treated patients from the Scandinavian Sarcoma Group Register. Eur J Cancer 36:710–716PubMedCrossRefGoogle Scholar
  160. Ueda T, Yoshikawa H, Mori S et al (1997) Influence of local recurrence on the prognosis of soft-tissue sarcomas. J Bone Joint Surg Br 79:553–557PubMedCrossRefGoogle Scholar
  161. Van Oosterom AT, Mouridsen HT, Nielsen S et al (2002) EORTC Soft Tissue and Bone Sarcoma Group. Results of randomised studies of the EORTC Soft Tissue and Bone Sarcoma Group (STBSG) with two different ifosfamide regimens in first-and second-line chemotherapy in advanced soft tissue sarcoma patients. Eur J Cancer 38:2397–2406PubMedCrossRefGoogle Scholar
  162. Vanel D, Lacombe MJ, Couanet D et al (1987) Musculoskeletal tumors follow-up with MR imaging after treatment with surgery and radiation therapy. Radiology 164:243–245PubMedGoogle Scholar
  163. Verhey LJ (1999) Comparison of three-dimensional conformal radiation therapy and intensity-modulated radiation therapy systems. Semin Radiat Oncol 9:78–98PubMedCrossRefGoogle Scholar
  164. Wanebo HJ, Temple WJ, Popp MB et al (1995) Preoperative regional therapy for extremity sarcoma. A tricenter update. Cancer 75:2299–2306PubMedCrossRefGoogle Scholar
  165. Weddington WW Jr, Segraves KB, Simon MA et al (1985) Psychological outcome of extremity sarcoma survivors undergoing amputation or limb salvage. J Clin Oncol 3:1393–1399PubMedGoogle Scholar
  166. Weiss SW, Goldblum JR (2001) Enzinger and Weiss’s soft tissue tumors. Mosby, PhiladelphiaGoogle Scholar
  167. White LM, Wunder JS, Bell RS et al (2005) Histologic assessment of peritumoral edema in soft tissue sarcoma. Int J Radiat Oncol Biol Phys 61:1439–1445PubMedCrossRefGoogle Scholar
  168. Williard WC, Hajdu SI, Casper ES et al (1992) Comparison of amputation with limb-sparing operations for adult soft tissue sarcoma of the extremity269. Ann Surg 215:389–396Google Scholar
  169. Wilson RR (1946) Radiological uses of fast protons. Radiology 47:487–491PubMedGoogle Scholar
  170. 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
  171. Yovine A, Riofrio M, Blay JY et al (2004) Phase II study of ecteinascidin-743 in advanced pretreated soft tissue sarcoma patients. J Clin Oncol 22:890–899PubMedCrossRefGoogle Scholar
  172. Zagars GK, Ballo MT (2003) Significance of dose in postoperative radiotherapy for soft tissue sarcoma. Int J Radiat Oncol Biol Phys 56:473–481PubMedCrossRefGoogle Scholar
  173. Zagars GK, Ballo MT, Pisters PW et al (2003a) Surgical margins and reresection in the management of patients with soft tissue sarcoma using conservative surgery and radiation therapy. Cancer 97:2544–2553PubMedCrossRefGoogle Scholar
  174. Zagars GK, Ballo MT, Pisters PW et al (2003b) Prognostic factors for patients with localized soft-tissue sarcoma treated with conservation surgery and radiation therapy: an analysis of 225 patients. Cancer 97:2530–2543PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2006

Authors and Affiliations

  • Thomas F. DeLaney
    • 1
  • David C. Harmon
    • 2
  • Andrew E. Rosenberg
    • 3
  • Francis J. Hornicek
    • 4
  1. 1.Northeast Proton Therapy CenterMassachusetts General Hospital, Harvard Medical SchoolBostonUSA
  2. 2.Hematology Oncology, Department of MedicineMassachusetts General Hospital, Harvard Medical SchoolBostonUSA
  3. 3.Surgical Pathology, Department of PathologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA
  4. 4.Orthopedic Oncology, Department of Orthopedic SurgeryMassachusetts General Hospital, Harvard Medical SchoolBostonUSA

Personalised recommendations