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Soft Tissue Sarcoma

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Book cover Cancer in Adolescents and Young Adults

Part of the book series: Pediatric Oncology ((PEDIATRICO))

Abstract

Soft tissue sarcomas represent a very heterogeneous group of mesenchymal malignant tumors that may occur at any age. Some histotypes are typically of a given age and rare in others, but as a whole group, soft tissue sarcomas are tumors bridging the pediatric and adult settings: adolescents and young adults are therefore age groups in which the soft tissue sarcoma family represents a subgroup of relatively frequent tumors. Managing these malignancies in patients in this age bracket poses various clinical problems, partly because different therapeutic approaches have been sometimes adopted by pediatric and adult oncologists, even though they were dealing with the same condition. In addition, whether the biology and clinical behavior of a given histotype is the same in patients of different ages remains to be seen. The treatment of adolescent and young adult patients with soft tissue sarcomas is particularly complex and necessarily w and requires adequate expertise. Cooperation between pediatric oncologists and adult oncologists is of critical importance to improve the quality of the treatment as well as the research programs dedicated to young patients with these diseases.

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References

  1. Kwong TN, Furtado S, Gerrand C (2014) What do we know about survivorship after treatment for extremity sarcoma? A systematic review. Eur J Surg Oncol 40(9):1109–1124

    Article  PubMed  Google Scholar 

  2. Ferrari A, Sultan I, Huang TT et al (2011) Soft tissue sarcoma across the age spectrum: a population-based study from the surveillance epidemiology and end results database. Pediatr Blood Cancer 57(6):943–949

    Article  PubMed  PubMed Central  Google Scholar 

  3. Corey RM, Swett K, Ward WG (2014) Epidemiology and survivorship of soft tissue sarcomas in adults: a national cancer database report. Cancer Med 3(5):1404–1415

    Article  PubMed  PubMed Central  Google Scholar 

  4. Hsieh MC, Wu XC, Andrews PA et al (2013) Racial and ethnic disparities in the incidence and trends of soft tissue sarcoma among adolescents and young adults in the United States, 1995–2008. J Adolesc Young Adult Oncol 2(3):89–94

    Article  PubMed  PubMed Central  Google Scholar 

  5. Borden EC, Baker LH, Bell RS et al (2003) Soft tissue sarcomas of adults: state of the translational science. Clin Cancer Res 9:1941–1956

    PubMed  Google Scholar 

  6. Chibon F, Lagarde P, Salas S et al (2009) Molecular signature of metastasis outcome in sarcomas. Proceedings of the 100th Annual Meeting of the American Association for Cancer Research, AACR, Denver/Philadelphia, 18–22 Apr 2009. Abstract nr 4983

    Google Scholar 

  7. Coindre JM, Chibon F (2010) Grading of soft tissue sarcomas – from histologic to molecular assessment. Companion Meeting of the International Society of Bone and Soft Tissue Pathology, Washington, DC, March 2010 www.uscap.org

  8. Fletcher CDM (2014) The evolving classification of soft tissue tumours – an update based on the new 2013 WHO classification. Histopathology 64:2–11

    Article  PubMed  Google Scholar 

  9. Guillou L, Coindre JM, Bonichon F et al (1997) Comparative study of the National Cancer Institute and French Federation of Cancer Centers Sarcoma Group grading systems in a population of 410 adult patients with soft tissue sarcoma. J Clin Oncol 15:350–362

    Article  CAS  PubMed  Google Scholar 

  10. Parham DM, Webber BL, Jenkins JJ et al (1995) Nonrahbdomyosarcomatous soft tissue sarcomas of childhood: formulation of a simplified system for grading. Mod Pathol 8:705–710

    CAS  PubMed  Google Scholar 

  11. Burningham Z, Hashibe M, Spector L et al (2012) The epidemiology of sarcoma. Clin Sarcoma Res 2(14):1–16

    Google Scholar 

  12. Ferrari A, Bisogno G, Macaluso A et al (2007) Soft tissue sarcomas in children and adolescents with neurofibromatosis type 1. Cancer 109(7):1406–1412

    Article  PubMed  Google Scholar 

  13. Sung L, Anderson JR, Arndt C et al (2004) Neurofibromatosis in children with Rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma study IV. J Pediatr 144(5):666–668

    Article  PubMed  Google Scholar 

  14. Kleinerman RA, Tucker MA, Abramson DH, Seddon JM, Tarone RE, Fraumeni JF Jr (2007) Risk of soft tissue sarcomas by individual subtype in survivors of hereditary retinoblastoma. J Natl Cancer Inst 99(1):24–31

    Article  PubMed  Google Scholar 

  15. Postow MA, Robson ME (2012) Inherited gastrointestinal stromal tumor syndromes: mutations, clinical features, and therapeutic implications. Clin Sarcoma Res 2(1):16

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Penel N, Grosjean J, Robin YM et al (2008) Frequency of certain established risk factors in soft tissue sarcomas in adults: a prospective descriptive study of 658 cases. Sarcoma 2008:459386

    Article  PubMed  PubMed Central  Google Scholar 

  17. Ferrari A, De Salvo GL, Oberlin O, Casanova M, De Paoli A, Rey A et al (2012) Synovial sarcoma in children and adolescents: a critical reappraisal of staging investigations in relation to the rate of metastatic involvement at diagnosis. Eur J Cancer 48:1370–1375

    Article  PubMed  Google Scholar 

  18. Charest M, Hickeson M, Lisbona R, Novales-Diaz JA, Derbekyan V, Turcotte RE (2009) FDG PET/CT imaging in primary osseous and soft tissue sarcomas: a retrospective review of 212 cases. Eur J Nucl Med Mol Imaging 36:1944–1951

    Article  PubMed  Google Scholar 

  19. Maurer HM, Beltangady M, Gehan EA et al (1988) The Inter- group Rhabdomyosarcoma Study I: a final report. Cancer 61:209–220

    Article  CAS  PubMed  Google Scholar 

  20. Harmer MH (1982) TNM Classification of pediatric tumors. UICC International Union Against Cancer, Geneva, pp 23–28

    Google Scholar 

  21. Wunder JS, Healey JH, Davis AM, Brennan MF (2000) A comparsion of staging systems for localized extremity soft tissue sarcoma. Cancer 88:2721–2730

    Article  CAS  PubMed  Google Scholar 

  22. Mathoulin-Pélissier S, Chevreau C, Bellera C et al (2014) Adherence to consensus-based diagnosis and treatment guidelines in adult soft-tissue sarcoma patients: a French prospective population-based study. Ann Oncol 25(1):225–231

    Article  PubMed  Google Scholar 

  23. Rossi CR, Vecchiato A, Mastrangelo G et al (2013) Adherence to treatment guidelines for primary sarcomas affects patient survival: a side study of the european CONnective TIssue CAncer NETwork (CONTICANET). Ann Oncol 24(6):1685–1691

    Article  CAS  PubMed  Google Scholar 

  24. Enneking WF, Spanier SS, Goodman MA (2003) A system for the surgical staging of musculoskeletal sarcoma. 1980. Clin Orthop Relat Res 415:4–18

    Article  Google Scholar 

  25. White LM, Wunder JS, Bell RS, O’Sullivan B, Catton C, Ferguson P, Blackstein M, Kandel RA (2005) Histologic assessment of peritumoral edema in soft tissue sarcoma. Int J Radiat Oncol Biol Phys 61(5):1439–1445

    Article  PubMed  Google Scholar 

  26. Haas RL, Delaney TF, O’Sullivan B, Keus RB, Le Pechoux C, Olmi P, Poulsen JP, Seddon B, Wang D (2012) Radiotherapy for management of extremity soft tissue sarcomas: why, when, and where? Int J Radiat Oncol Biol Phys 84(3):572–580

    Article  PubMed  Google Scholar 

  27. Baldini EH, Goldberg J, Jenner C, Manola JB, Demetri GD, Fletcher CD, Singer S (1999) Long-term outcomes after function-sparing surgery without radiotherapy for soft tissue sarcoma of the extremities and trunk. J Clin Oncol 17(10):3252–3259

    Article  CAS  PubMed  Google Scholar 

  28. Geer RJ, Woodruff J, Casper ES, Brennan MF (1992) Management of small soft-tissue sarcoma of the extremity in adults. Arch Surg 127(11):1285–1289

    Article  CAS  PubMed  Google Scholar 

  29. O’Sullivan B, Davis AM, Turcotte R, Bell R, Catton C, Chabot P, Wunder J, Kandel R, Goddard K, Sadura A, Pater J, Zee B (2002) Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised trial. Lancet 359(9325):2235–2241

    Article  PubMed  Google Scholar 

  30. Davis AM, O’Sullivan B, Bell RS, Turcotte R, Catton CN, Wunder JS, Chabot P, Hammond A, Benk V, Isler M, Freeman C, Goddard K, Bezjak A, Kandel RA, Sadura A, Day A, James K, Tu D, Pater J, Zee B (2002) Function and health status outcomes in a randomized trial comparing preoperative and postoperative radiotherapy in extremity soft tissue sarcoma. J Clin Oncol 20(22):4472–4477

    Article  CAS  PubMed  Google Scholar 

  31. O’Sullivan B, Griffin AM, Dickie CI, Sharpe MB, Chung PW, Catton CN, Ferguson PC, Wunder JS, Deheshi BM, White LM, Kandel RA, Jaffray DA, Bell RS (2013) 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 119(10):1878–1884

    Article  PubMed  Google Scholar 

  32. Griffin AM, Euler CI, Sharpe MB, Ferguson PC, Wunder JS, Bell RS, Chung PW, Catton CN, O’Sullivan B (2007) Radiation planning comparison for superficial tissue avoidance in radiotherapy for soft tissue sarcoma of the lower extremity. Int J Radiat Oncol Biol Phys 67(3):847–856

    Article  PubMed  Google Scholar 

  33. Yang JC, Chang AE, Baker AR, Sindelar WF, Danforth DN, Topalian SL, DeLaney T, Glatstein E, Steinberg SM, Merino MJ, Rosenberg SA (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(1):197–203

    Article  CAS  PubMed  Google Scholar 

  34. Pisters PW, Harrison LB, Leung DH, Woodruff JM, Casper ES, Brennan MF (1996) Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. J Clin Oncol 14(3):859–868

    Article  CAS  PubMed  Google Scholar 

  35. O’Donnell PW, Griffin AM, Eward WC, Sternheim A, Catton CN, Chung PW, O’Sullivan B, Ferguson PC, Wunder JS (2014) The effect of the setting of a positive surgical margin in soft tissue sarcoma. Cancer 120(18):2866–2875

    Article  PubMed  Google Scholar 

  36. Gerrand CH, Wunder JS, Kandel RA, O’Sullivan B, Catton CN, Bell RS, Griffin AM, Davis AM (2001) Classification of positive margins after resection of soft-tissue sarcoma of the limb predicts the risk of local recurrence. J Bone Joint Surg Br 83(8):1149–1155

    Article  CAS  PubMed  Google Scholar 

  37. Trans-Atlantic RPS Working Group (2015) Management of primary retroperitoneal sarcoma (RPS) in the adult: a consensus approach from the Trans-Atlantic RPS Working Group. Ann Surg Oncol 22(1):256–263

    Article  Google Scholar 

  38. Tukenova M, Guibout C, Hawkins M, Quiniou E, Mousannif A, Pacquement H, Winter D, Bridier A, Lefkopoulos D, Oberlin O, Diallo I, de Vathaire F (2011) Radiation therapy and late mortality from second sarcoma, carcinoma, and hematological malignancies after a solid cancer in childhood. Int J Radiat Oncol Biol Phys 80(2):339–346

    Article  PubMed  Google Scholar 

  39. Newton WA, Gehan EA, Webber BL et al (1995) Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification – an Intergroup Rhabdomyosarcoma Study. Cancer 70:1073–1085

    Article  Google Scholar 

  40. Davicioni E, Anderson MJ, Finckenstein FG et al (2009) Molecular classification of rhabdomyosarcoma – genotypic and phenotypic determinants of diagnosis: a report from the Children’s Oncology Group. Am J Pathol 174(2):550–564

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  41. Williamson D, Missiaglia E, de Reynies A et al (2010) Fusion gene-negative alveolar rhabdomyosarcoma is clinically and molecularly indistinguishable from embryonal rhabdomyosarcoma. J Clin Oncol 28(13):2151–2158

    Article  PubMed  Google Scholar 

  42. Crist WM, Anderson JR, Meza JL et al (2001) Inter- group Rhabdomyosarcoma Study-IV: results for patients with nonmetastatic disease. J Clin Oncol 19:3091–3102

    Article  CAS  PubMed  Google Scholar 

  43. Crist WM, Garnsey L, Beltangady MS et al (1990) Prognosis in children with rhabdomyosarcoma: a report of the Intergroup Rhabdomyosarcoma Studies I and II. J Clin Oncol 8:443–452

    Article  CAS  PubMed  Google Scholar 

  44. Raney RB, Anderson JR, Barr FG et al (2003) Rhabdomyosar- coma and undifferentiated sarcoma in first two decades of life: a lelective review of Intergroup Rhabdomyosar- coma Study Group experience and rationale for Inter- group Rhabdomyosarcoma Study V. J Pediatr Oncol 41:1–6

    Article  Google Scholar 

  45. Stevens MC, Rey A, Bouvet N et al (2005) Treatment of nonmetastatic rhabdomyosarcoma in childhood and adolescence: third study of the International Society of Paediatric Oncology--SIOP Malignant Mesenchymal Tumor 89. J Clin Oncol 23(12):2618–2628

    Google Scholar 

  46. Arndt CA, Stoner JA, Hawkins DS et al (2009) Vincristine, actinomycin, and cyclophosphamide compared with vincristine, actinomycin, and cyclophosphamide alternating with vincristine, topotecan, and cyclophosphamide for intermediate-risk rhabdomyosarcoma: Children’s Oncology Group Study D9803. J Clin Oncol 27:5182–5188

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  47. Oberlin O, Rey A, Sanchez de Toledo J et al (2012) Randomized comparison of intensified six-drug versus standard three-drug chemotherapy for high-risk nonmetastatic rhabdomyosarcoma and other chemotherapy-sensitive childhood soft tissue sarcomas: long-term results from the International Society of Pediatric Oncology MMT95 study. J Clin Oncol 30(20):2457–2465

    Article  CAS  PubMed  Google Scholar 

  48. Ferrari A, Casanova M (2005) Current chemotherapeutic strategies for rhabdomyosarcoma. Expert Rev Anticancer Ther 5(2):283–294

    Article  CAS  PubMed  Google Scholar 

  49. Sultan I, Ferrari A (2010) Selecting multimodal therapy for rhabdomyosarcoma. Expert Rev Anticancer Ther 10(8):1285–1301

    Article  PubMed  Google Scholar 

  50. Joshi D, Anderson JR, Paidas C et al (2004) Age is an independent prognostic factor in rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Children’s Oncology Group. Pediatr Blood Cancer 42:64–73

    Article  CAS  PubMed  Google Scholar 

  51. Stevens MC (2005) Treatment for childhood rhabdomyosarcoma: the cost of cure. Lancet Oncol 6(2):77–84

    Article  PubMed  Google Scholar 

  52. Bisogno G, Ferrari A, Bergeron C et al (2005) The ifosfamide, vincristine, actinomycin, doxorubicin (IVADo) regimen, an intensified chemotherapy for children with soft tissue sarcoma. A pilot study by the European pediatric Soft Tissue sarcoma Study Group. Cancer 103:1719–1724

    Article  CAS  PubMed  Google Scholar 

  53. Bisogno G, De Salvo GL, Bergeron C et al (2014) The role of doxorubicin in the treatment of rhabdomyosarcoma: preliminary results from the EpSSG RMS 2005 randomized trial. Pediatr Blood Cancer 61(S2):S105–S433, 46th Congress of the International Society of Paediatric Oncology (SIOP) 2014 (October 2014), O-105

    Article  Google Scholar 

  54. Casanova M, Ferrari A, Bisogno G et al (2004) Vinorelbine and low-dose cyclophosphamide in pediatric sarcomas: pilot study for the future European Rhabdomyosarcoma Protocol. Cancer 101:1664–1671

    Article  CAS  PubMed  Google Scholar 

  55. Hawkins DS, Anderson JR, Mascarenhas L et al (2014) Vincristine, dactinomycin, cyclophosphamide (VAC) versus VAC/V plus irinotecan (VI) for intermediate-risk rhabdomyosarcoma (IRRMS): a report from the Children’s Oncology Group Soft Tissue Sarcoma Committee. J Clin Oncol 32(15_suppl (May 20 Suppl)):10004, 2014 ASCO Annual Meeting Abstracts

    Google Scholar 

  56. Oberlin O, Rey A, Lyden E et al (2008) Prognostic factors in metastatic rhabdomyosarcomas: results of a pooled analysis from United States and European cooperative groups. J Clin Oncol 26(14):2384–2389

    Article  PubMed  PubMed Central  Google Scholar 

  57. Bisogno G, Ferrari A, Prete A et al (2009) Sequential high-dose chemotherapy for children with metastatic rhabdomyosarcoma. Eur J Cancer 45(17):3035–3041

    Article  CAS  PubMed  Google Scholar 

  58. Bisogno G, Compostella A, Ferrari A et al (2012) Rhabdomyosarcoma in adolescents: a report from the AIEOP Soft Tissue Sarcoma Committee. Cancer 118(3):821–827

    Article  PubMed  Google Scholar 

  59. Ferrari A, Miceli R, Casanova M et al (2010) The symptom interval in children and adolescents with soft tissue sarcomas. Cancer 116:177–183

    PubMed  Google Scholar 

  60. Esnaola NF, Rubin BP, Baldini EH et al (2001) Response to chemotherapy and predictors of survival in adult rhabdomyosarcoma. Cancer 91:794–803

    Article  Google Scholar 

  61. Little DJ, Ballo MT, Zagars GK et al (2002) Adult rhabdomyosarcoma: outcome following multimodality treatment. Cancer 95:377–388

    Article  PubMed  Google Scholar 

  62. Ferrari A, Dileo P, Casanova M et al (2003) Rhabdomyosarcoma in adults. A retrospective analysis of 171 patients treated at a single institution. Cancer 98:571–580

    Article  PubMed  Google Scholar 

  63. Sultan I, Qaddoumi I, Yaser S, Rodriguez-Galindo C, Ferrari A (2009) Comparing adult and pediatric rhabdomyosarcoma in the surveillance, epidemiology and end results program, 1973 to 2005: an analysis of 2,600 patients. J Clin Oncol 27(20):3391–3397

    Article  PubMed  Google Scholar 

  64. Ferrari A, Bisogno G, Meazza C et al (2012) The challenge of access to care for soft tissue sarcomas bridging pediatric and adult age: the Italian pediatric oncology view. Expert Rev Anticancer Ther 12(2):243–254

    Article  PubMed  Google Scholar 

  65. Ferrari A, Casanova M (2005) New concepts for the treatment of pediatric non-rhabdomyosarcoma soft tissue sarcomas. Expert Rev Anticancer Ther 5(2):307–318

    Article  CAS  PubMed  Google Scholar 

  66. 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 101:627–634

    Article  PubMed  Google Scholar 

  67. Okcu MF, Munsell M, Treuner J et al (2003) Synovial sarcoma of childhood and adolescence: a multicenter, multivariate analysis of outcome. J Clin Oncol 21:1602–1611

    Article  PubMed  Google Scholar 

  68. Brecht IB, Ferrari A, Int-Veen C et al (2006) Grossly-resected synovial sarcoma treated by the German and Italian pediatric soft tissue sarcoma cooperative group: discussion on the role of adjuvant therapies. Pediatr Blood Cancer 46:11–17

    Article  PubMed  Google Scholar 

  69. Ferrari A, Bisogno G, Alaggio G et al (2008) Synovial sarcoma of children and adolescents: the prognostic role of axial sites. Eur J Cancer 44:1202–1209

    Article  PubMed  Google Scholar 

  70. Brennan B, Stevens M, Kelsey A, Stiller CA (2010) Synovial sarcoma in childhood and adolescence: a retrospective series of 77 patients registered by the Children’s Cancer and Leukaemia Group between 1991 and 2006. Pediatr Blood Cancer 55:85–90

    PubMed  Google Scholar 

  71. Orbach D, Dowell HM, Rey A et al (2011) Sparing strategy does not compromise prognosis in pediatric localized synovial sarcoma: experience of the International Society of Pediatric Oncology, Malignant Mesenchymal Tumors (SIOP-MMT) Working Group. Pediatr Blood Cancer 57:1130–1136

    Article  PubMed  Google Scholar 

  72. Ferrari A, De Salvo GL, Dall’Igna P et al (2012) Salvage rates and prognostic factors after relapse in children and adolescents with initially localised synovial sarcoma. Eur J Cancer 48:3448–3455

    Article  PubMed  Google Scholar 

  73. Stanelle EJ, Christison-Lagay ER (2013) Pediatric and adolescent synovial sarcoma: multivariate analysis of prognostic factors and survival outcomes. Ann Surg Oncol 20:73–79

    Article  PubMed  Google Scholar 

  74. Lewis JJ, Antonescu CR, Leung DH et al (2000) Synovial sarcoma: a multivariate analysis of prognostic factors in 112 patients with primary localized tumours of the extremity. J Clin Oncol 18:2087–2094

    Article  CAS  PubMed  Google Scholar 

  75. Trassard M, Le Doussal V, Hacene K et al (2001) Prognostic factors in localized primary synovial sarcoma: a multicenter study of 128 adult patients. J Clin Oncol 19:525–534

    Article  CAS  PubMed  Google Scholar 

  76. Spurrell EL, Fisher C, Thomas JM, Judson IR (2005) Prognostic factors in advanced synovial sarcoma: an analysis of 104 patients treated at the Royal Marsden Hospital. Ann Oncol 16(3):437–444

    Article  CAS  PubMed  Google Scholar 

  77. Eilber FC, Brennan MF, Eilber FR et al (2007) Chemotherapy is associated with improved survival in adult patients with primary extremity synovial sarcoma. Ann Surg 246(1):105–113

    Article  PubMed  PubMed Central  Google Scholar 

  78. Guadagnolo BA, Zagars GK, Ballo MT et al (2007) Long-term outcomes for synovial sarcoma treated with conservation surgery and radiotherapy. Int J Radiat Oncol Biol Phys 69(4):1173–1180

    Article  PubMed  Google Scholar 

  79. Canter RJ, Qin LX, Maki RG et al (2008) A synovial sarcoma-specific preoperative nomogram supports a survival benefit to ifosfamide-based chemotherapy and improves risk stratification for patients. Clin Cancer Res 14(24):8191–8197

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  80. Italiano A, Penel N, Robin YM et al (2009) Neo/adjuvant chemotherapy does not improve outcome in resected primary synovial sarcoma: a study of the French Sarcoma Group. Ann Oncol 20(3):425–430

    Article  CAS  PubMed  Google Scholar 

  81. Palmerini E, Staals EL, Alberghini M et al (2009) Synovial sarcoma: retrospective analysis of 250 patients treated at a single institution. Cancer 115(13):2988–2998

    Article  PubMed  Google Scholar 

  82. Al-Hussaini H, Hogg D, Blackstein ME et al (2011) Clinical features, treatment, and outcome in 102 adult and pediatric patients with localized high-grade synovial sarcoma. Sarcoma 2011:231789

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  83. Sultan I, Rodriguez-Galindo C, Saab R et al (2009) Comparing children and adults with synovial sarcoma in the Surveillance, Epidemiology and End Results Program, 1983 to 2005: an analysis of 1268 patients. Cancer 115:3537–3547

    Article  PubMed  Google Scholar 

  84. Gronchi A, Casali P (2013) Adjuvant therapy for high-risk soft tissue sarcoma in the adult. Curr Treat Options Oncol 14(3):415–424

    Article  PubMed  Google Scholar 

  85. Ferrari A, Chiaravalli S, Casanova M, Gasparini P, Corradini N, Orbach D. Considering chemotherapy in synovial sarcoma. Expert Opin Orphan Drugs, (in press)

    Google Scholar 

  86. Ferrari A, De Salvo GL, Brennan B et al (2015) Synovial sarcoma in children and adolescents: the European pediatric Soft tissue sarcoma Study Group prospective trial (EpSSG NRSTS 2005). Ann Oncol 26:567–572

    Article  CAS  PubMed  Google Scholar 

  87. Ferrari A, Casanova M (2013) Relapse in synovial sarcoma: what can be done for poor outcomes? Clin Pract 10(4):389–391

    Article  CAS  Google Scholar 

  88. Albritton KH, Randall RL (2005) Prospects for targeted therapy of synovial sarcoma. J Pediatr Hematol Oncol 27:219–222

    Article  PubMed  Google Scholar 

  89. Lagarde P, Przybyl J, Brulard C et al (2013) Chromosome instability accounts for reverse metastatic outcomes of pediatric and adult synovial sarcomas. J Clin Oncol 31:608–615

    Article  CAS  PubMed  Google Scholar 

  90. Chakiba C, Lagarde P, Pissaloux D et al (2014) Response to chemotherapy is not related to chromosome instability in synovial sarcoma. Ann Oncol 25(11):2267–2271

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  91. Spunt SL, Poquette CA, Hurt YS et al (1999) Prognostic factors for children and adolescents with surgically resected nonrhabdomyosarcoma soft tissue sarcoma: an analysis of 121 patients treated at St Jude Children’s Research Hospital. J Clin Oncol 17:3697–3705

    Article  CAS  PubMed  Google Scholar 

  92. Ferrari A, Casanova M, Collini P et al (2005) Adult-type soft tissue sarcomas in pediatric age: experience at the Istituto Nazionale Tumori in Milan. J Clin Oncol 23:4021–4030

    Article  PubMed  Google Scholar 

  93. Ferrari A, Miceli R, Rey A et al (2011) Non-metastatic unresected pediatric non-rhabdomyosarcoma soft tissue sarcomas: results of a pooled analysis from United States and European groups. Eur J Cancer 47:724–731

    Article  PubMed  Google Scholar 

  94. Soft tissue sarcoma (2010). In: Edge SB, Byrd DR, Compton CC, et al (eds) AJCC cancer staging manual, 7th edn. Springer, New York, pp 291–296

    Google Scholar 

  95. 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 911 consecutive patients treated at a single institution. J Clin Oncol 23:96–104

    Article  CAS  PubMed  Google Scholar 

  96. Stojadinovic A, Leung DHY, Hoos A et al (2002) Analysis of the prognostic significance of microscopic margins in 2084 localized primary adult soft tissue sarcomas. Ann Surg 235:424–443

    Article  PubMed  PubMed Central  Google Scholar 

  97. Ferrari A, Miceli R, Casanova M et al (2007) Adult-type soft tissue sarcomas in pediatric age: a nomogram-based prognostic comparison with adult sarcomas. Eur J Cancer 43:2691–2697

    Article  PubMed  Google Scholar 

  98. Patel SR, Vadhan-Raj S, Burgess MA et al (1998) Results of two consecutive trials of dose-intensive chemotherapy with doxorubicin and ifosfamide in patients with sarcomas. Am J Clin Oncol 21:317–321

    Article  CAS  PubMed  Google Scholar 

  99. Judson I, Verweij J, Gelderblom H et al (2014) Doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced or metastatic soft-tissue sarcoma: a randomised controlled phase 3 trial. Lancet Oncol 15:415–423

    Article  CAS  PubMed  Google Scholar 

  100. Maki RG, Wathen JK, Patel SR et al (2007) Randomized phase II study of gemcitabine and docetaxel compared with gemcitabine alone in patients with metastatic soft tissue sarcomas: results of sarcoma alliance for research through collaboration study 002. J Clin Oncol 25:2755–2763

    Article  CAS  PubMed  Google Scholar 

  101. Garcia del Muro J, López-Pousa A, Maurel J et al (2011) Randomized phase II study comparing gemcitabine plus dacarbazine versus dacarbazine alone in patients with previously treated soft tissue sarcoma: a Spanish Group for Research on Sarcomas study. J Clin Oncol 29:2528–2533

    Article  CAS  PubMed  Google Scholar 

  102. Samuels BL, Chawla S, Patel S et al (2013) Clinical outcomes and safety with trabectedin therapy in patients with advanced soft tissue sarcomas following failure of prior chemotherapy: results of a worldwide expanded access program study. Ann Oncol 24(6):1703–1709

    Article  CAS  PubMed  Google Scholar 

  103. Sarcoma Meta-analysis Collaboration (1997) Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Lancet 350:1647–1654

    Article  Google Scholar 

  104. Pervaiz N, Colterjohn N, Farrokhyar F et al (2008) A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer 113:573–581

    Article  PubMed  Google Scholar 

  105. Frustaci S, Gherlinzoni F, De Paoli A et al (2001) Adjuvant chemotherapy for adult soft tissue sarcomas of extremities and girdles: results of the Italian randomized cooperative trial. J Clin Oncol 19:1238–1247

    Article  CAS  PubMed  Google Scholar 

  106. Woll P, Reichardt P, Le Cesna A et al (2012) Adjuvant chemotherapy with doxorubicin, ifosfamide, and lenograstim for resected soft-tissue sarcoma (EORTC 62931): a multicentre randomised controlled trial. Lancet Oncol 13:1045–1054

    Article  CAS  PubMed  Google Scholar 

  107. von Mehren M (2011) J Clin Oncol 29, abst 10016

    Google Scholar 

  108. De Matteo RP, Ballman KV, Antonescu CR et al (2009) Adjuvant imatinib mesylate after resection of localised, primary gastrointestinal stromal tumour: a randomised, double-blind, placebo-controlled trial. Lancet 373:1097–1104

    Article  CAS  Google Scholar 

  109. Joensuu H, Eriksson M, Sundby Hall K et al (2012) One vs three years of adjuvant imatinib for operable gastrointestinal stromal tumor: a randomized trial. JAMA 307:1265–1272

    Article  CAS  PubMed  Google Scholar 

  110. Demetri GD, van Oosterom AT, Garrett CR et al (2006) Efficacy and safety of sunitinib in patients with advanced gastrointestinal stromal tumour after failure of imatinib: a randomised controlled trial. Lancet 368:1329–1338

    Article  CAS  PubMed  Google Scholar 

  111. Demetri GD, Reichardt P, Kang YK, Blay JY et al (2013) Efficacy and safety of regorafenib for advanced gastrointestinal stromal tumours after failure of imatinib and sunitinib (GRID): an international, multicentre, randomised, placebo-controlled, phase 3 trial. Lancet 381:295–302

    Article  CAS  PubMed  Google Scholar 

  112. McArthur GA, Demetri GD, van Oosterom A et al (2005) Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with imatinib: Imatinib Target Exploration Consortium Study B2225. J Clin Oncol 23(4):866–873

    Article  CAS  PubMed  Google Scholar 

  113. Stacchiotti S, Negri T, Zaffaroni N et al (2011) Sunitinib in advanced alveolar soft part sarcoma: evidence of a direct antitumor effect. Ann Oncol 22:1682–1690

    Article  CAS  PubMed  Google Scholar 

  114. Kummar S, Allen D, Monks A et al (2013) Cediranib for metastatic alveolar soft part sarcoma. J Clin Oncol 31:2296–2302

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  115. Stacchiotti S, Negri T, Libertini M et al (2012) Sunitinib malate in solitary fibrous tumor (SFT). Ann Oncol 23:3171–3179

    Article  CAS  PubMed  Google Scholar 

  116. Park MS, Patel SR, Ludwig JA et al (2011) Activity of temozolomide and bevacizumab in the treatment of locally advanced, recurrent, and metastatic hemangiopericytoma and malignant solitary fibrous tumor. Cancer 117:4939–4947

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  117. Wagner AJ, Malinowska-Kolodziej I, Morgan JA et al (2010) Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors. J Clin Oncol 28:835–840

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  118. Butrynski JE, D’Adamo DR, Hornick JL et al (2010) Crizotinib in ALK-rearranged inflammatory myofibroblastic tumor. N Engl J Med 363:1727–1733

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  119. van der Graaf WT, Blay JY, Chawla SP et al (2012) Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet 379:1879–1886

    Article  PubMed  CAS  Google Scholar 

  120. Miettinen MLJ (2006) Gastrointestinal stromal tumors. Review of morphology, molecular pathology, prognosis and differential diagnosis. Arch Pathol Lab Med 130:1466–1478

    CAS  PubMed  Google Scholar 

  121. Novelli M, Rossi S, Rodriguez-Justo M et al (2010) DOG1 and CD117 are the antibodies of choice in the diagnosis of gastrointestinal stromal tumors. Histopathology 57:259–270

    Article  PubMed  Google Scholar 

  122. Heinrich MC, Corless CL, Demetri GD et al (2003) Kinase mutations and imatinib response in patients with metastatic gastrointestinal stromal tumor. J Clin Oncol 21(23):4342–4349

    Article  CAS  PubMed  Google Scholar 

  123. Miettinen M, Lasota J (2006) Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol 23:70–83

    Article  PubMed  Google Scholar 

  124. Gold JS, Gönen M, Gutierrez A et al (2009) Development and validation of a prognostic nomogram for recurrence-free survival after complete surgical resection of localised primary gastrointestinal stromal tumour: a retrospective analysis. Lancet Oncol 10:1045–1052

    Article  PubMed  PubMed Central  Google Scholar 

  125. ESMO/European Sarcoma Network Working Group (2014) Gastrointestinal stromal tumours: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 25(Suppl 3):iii21–iii26

    Article  Google Scholar 

  126. Dematteo RP, Ballman KV, Antonescu CR et al (2009) Adjuvant imatinib mesylate after resection of localised, primary gastrointestinal stromal tumor: a randomised, double-blind, placebo-controlled trial. Lancet 373:1097–1104

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  127. Gronchi A, Judson I, Nishida T et al (2009) Adjuvant treatment of GIST with imatinib: solid ground or still quicksand? A comment on behalf of the EORTC Soft Tissue and Bone Sarcoma Group, the Italian Sarcoma Group, the NCRI Sarcoma Clinical Studies Group (UK), the Japanese Study Group on GIST, the French Sarcoma Group and the Spanish Sarcoma Group (GEIS). Eur J Cancer 45:1103–1106

    Article  PubMed  Google Scholar 

  128. Eisenberg BL, Harris J, Blanke CD et al (2009) Phase II trial of neoadjuvant/adjuvant imatinib mesylate (IM) for advanced primary and metastatic/recurrent operable gastrointestinal stromal tumor (GIST): early results of RTOG 0132/ACRIN 6665. J Surg Oncol 99:42–47

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  129. Blanke CD, Demetri GD, von Mehren M et al (2008) Long-term results from a randomized phase II trial of standard- versus higher-dose imatinib mesylate forpatients with unresectable or metastatic gastrointestinal stromal tumors expressing KIT. J Clin Oncol 26:620–625

    Article  CAS  PubMed  Google Scholar 

  130. Blanke CD, Rankin C, Demetri GD et al (2008) Phase III randomized, intergroup trial assessing imatinib mesylate at two dose levels in patients with unresectable or metastatic gastrointestinal stromal tumors expressing the kit receptor tyrosine kinase: S0033. J Clin Oncol 26:626–632

    Article  CAS  PubMed  Google Scholar 

  131. Gastrointestinal Stromal Tumor Meta-Analysis Group (MetaGIST) (2010) Comparison of two doses of imatinib for the treatment of unresectable or metastatic gastrointestinal stromal tumors: a meta-analysis of 1640 patients. J Clin Oncol 28:1247–1253

    Article  CAS  Google Scholar 

  132. Le Cesne A, Ray-Coquard I, Bui BN et al (2010) Discontinuation of imatinib in patients with advanced gastrointestinal stromal tumors after 3 years of treatment: an openlabel multicentre randomised phase 3 trial. Lancet Oncol 11:942–949

    Article  PubMed  CAS  Google Scholar 

  133. Demetri GD, Reichardt P, Kang YK et al.; on behalf of all GRID study investigators (2013) Efficacy and safety of regorafenib for advanced gastrointestinal stromal tumours after failure of imatinib and sunitinib (GRID): an international, multicentre, randomised, placebo-controlled, phase 3 trial. Lancet 381:295–302

    Google Scholar 

  134. Benesch M, Wardelmann E, Ferrari A et al (2009) Gastrointestinal stromal tumors (GIST) in children and adolescents: a comprehensive review of the current literature. Pediatr Blood Cancer 53:1171–1179

    Article  PubMed  Google Scholar 

  135. Price VE, Zielenska M, Chilton-MacNeill S, Smith CR, Pappo AS (2005) Clinical and molecular characteristics of pediatric gastrointestinal stromal tumors (GISTs). Pediatr Blood Cancer 45(1):20–24

    Article  PubMed  Google Scholar 

  136. Pappo AS, Janeway KA (2009) Pediatric gastrointestinal stromal tumors. Hematol Oncol Clin North Am 23:15–34

    Article  PubMed  Google Scholar 

  137. Miettinen M, Lasota J, Sobin LH (2005) Gastrointestinal stromal tumors of the stomach in children and young adults: a clinicopathologic, immunohistochemical, and molecular genetic study of 44 cases with long-term follow-up and review of the literature. Am J Surg Pathol 29(10):1373–1381

    Article  PubMed  Google Scholar 

  138. Janeway KA, Liegl B, Harlow B et al (2007) Pediatric KIT–wild-type and platelet-derived growth factor receptor alpha–wild-type gastrointestinal stromal tumors share KIT activation but not mechanisms of genetic progression with adult gastrointestinal stromal tumors. Cancer Res 67:9084–9088

    Article  CAS  PubMed  Google Scholar 

  139. Janeway KA, Zhu MJ, Barretina J et al (2010) Strong expression of IGF1R in pediatric gastrointestinal stromal tumors without IGF1R genomic amplification. Int J Cancer 127(11):2718–2722

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  140. Miettinen M, Fetsch JF, Sobin LH, Lasota J (2006) Gastrointestinal stromal tumors in patients with neurofibromatosis 1: a clinicopathologic and molecular genetic study of 45 cases. Am J Surg Pathol 30(1):90–96

    Article  PubMed  Google Scholar 

  141. Zhang L, Smyrk TC, Young WF Jr et al (2010) Gastric stromal tumors in Carney triad are different clinically, pathologically, and behaviorally from sporadic gastric gastrointestinal stromal tumors: findings in 104 cases. Am J Surg Pathol 34:53–64

    Article  PubMed  PubMed Central  Google Scholar 

  142. Janeway KA, Albritton KH, Van Den Abbeele AD et al (2009) Sunitinib treatment in pediatric patients with advanced GIST following failure of imatinib. Pediatr Blood Cancer 52(7):767–771

    Article  PubMed  Google Scholar 

  143. Janeway KA, Pappo A (2012) Treatment guidelines for gastrointestinal stromal tumors in children and young adults. J Pediatr Hematol Oncol 34(Suppl 2):S69–S72

    Article  CAS  PubMed  Google Scholar 

  144. Pappo AS, Janeway K, Laquaglia M, Kim SY (2011) Special considerations in pediatric gastrointestinal tumors. J Surg Oncol 104(8):928–932

    Article  PubMed  Google Scholar 

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Ferrari, A., Patel, S.R., Wunder, J., Albritton, K.H. (2017). Soft Tissue Sarcoma. In: Bleyer, A., Barr, R., Ries, L., Whelan, J., Ferrari, A. (eds) Cancer in Adolescents and Young Adults. Pediatric Oncology. Springer, Cham. https://doi.org/10.1007/978-3-319-33679-4_15

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