The Non-rhabdomyosarcoma Soft Tissue Sarcomas, Desmoid Tumor and Osteosarcoma

  • Christopher L. Tinkle
  • John T. LucasJr.
Part of the Pediatric Oncology book series (PEDIATRICO)


Soft-tissue sarcoma (STS) are a group of rare tumors diagnosed in children and adults that require multi-modality specialty care. The etiology of STS in children is largely unknown although they are among the tumor types associated with cancer predisposition syndromes and included in the differential of radiation-induced malignancies. STS include a broad variety of histologic subtypes, diverse presentations, and wide-ranging lethality. Evaluation and management of STS in pediatric patients has evolved based on knowledge derived from clinical trials performed in adults and more recently prospective studies performed exclusively with children. Surgery, irradiation modalities, and conventional chemotherapy have identified roles in the treatment of pediatric patients with current studies focused on toxicity reduction in low-risk patients, optimal sequencing of therapy and new agent testing in intermediate and high-risk patients, and systematic salvage strategies for patients that do not respond to front-line therapy.


  1. Edge SB, Byrd DR, Compton CC et al (eds) (2010) AJCC cancer staging manual, 7th edn. Springer, New YorkGoogle Scholar
  2. Abbas JS, Holyoke ED, Moore R, Karakousis CP (1981) The surgical treatment and outcome of soft-tissue sarcoma. Arch Surg 116:765–769PubMedGoogle Scholar
  3. Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Sarcoma Meta-analysis Collaboration. Lancet 350(9092):1647–1654, 1997Google Scholar
  4. Alaggio R, Coffin CM (2015) The Evolution of Pediatric Soft Tissue Sarcoma Classification in the Last 50 Years. Pediatr Dev Pathol 18:481–494PubMedGoogle Scholar
  5. Alcorn KM, Deans KJ, Congeni A, Sulkowski JP, Bagatell R, Mattei P, Minneci PC (2013) Sentinel lymph node biopsy in pediatric soft tissue sarcoma patients: utility and concordance with imaging. J Pediatr Surg 48:1903–1906PubMedGoogle Scholar
  6. Alvegard TA, Berg NO (1989) Histopathology peer review of high-grade soft tissue sarcoma: the Scandinavian Sarcoma Group experience. J Clin Oncol 7:1845–1851PubMedGoogle Scholar
  7. Andrassy RJ (2002) Advances in the surgical management of sarcomas in children. Am J Surg 184:484–491PubMedGoogle Scholar
  8. Antman K, Crowley J, Balcerzak SP, Rivkin SE, Weiss GR, Elias A, Natale RB, Cooper RM, Barlogie B, Trump DL et al (1993) An intergroup phase III randomized study of doxorubicin and dacarbazine with or without ifosfamide and mesna in advanced soft tissue and bone sarcomas. J Clin Oncol 11:1276–1285PubMedGoogle Scholar
  9. Bahig H, Roberge D, Bosch W, Levin W, Petersen I, Haddock M, Freeman C, Delaney TF, Abrams RA, Indelicato DJ, Baldini EH, Hitchcock Y, Kirsch DG, Kozak KR, Wolfson A, Wang D (2013) Agreement among RTOG sarcoma radiation oncologists in contouring suspicious peritumoral edema for preoperative radiation therapy of soft tissue sarcoma of the extremity. Int J Radiat Oncol Biol Phys 86:298–303PubMedPubMedCentralGoogle Scholar
  10. Baker LH, Wathen K, Chugh R, Thomas D, Thall PF, Maki RG, Samuels BL, Meyers PA, Priebat DA, Benjamin RS (2004) Activity of imatinib mesylate in desmoid tumors: interim analysis of a Sarcoma Alliance for Research thru Collaboration (SARC) phase II trial. ASCO Meeting Abstract Sep 3, 2004: 9013. J Clin Oncol 22(14S)Google Scholar
  11. 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 17:3252–3259PubMedGoogle Scholar
  12. Barker JL Jr, Paulino AC, Feeney S, Mcculloch T, Hoffman H (2003) Locoregional treatment for adult soft tissue sarcomas of the head and neck: an institutional review. Cancer J 9:49–57PubMedGoogle Scholar
  13. Baumert BG, Spahr MO, von Hochstetter A, Beauvois S, Landmann C, Fridrich K, Villa S, Kirschner MJ, Storme G, Thum P, Streuli HK, Lombriser N, Maurer R, Ries G, Bleher EA, Willi A, Allemann J, Buehler U, Blessing H, Luetolf UM, Davis JB, Seifert B, Infanger M (2007) The impact of radiotherapy in the treatment of desmoid tumours. An international survey of 110 patients. A study of the Rare Cancer Network. Radiat Oncol 2:12PubMedPubMedCentralGoogle Scholar
  14. Ben Arush MW, Nahum MP, Meller I, Ben Itzhak O, Kuten A, El Hassid R, Linn S (1999) The role of chemotherapy in childhood soft tissue sarcomas other than rhabdomyosarcomas: experience of the Northern Israel Oncology Center. Pediatr Hematol Oncol 16:397–406PubMedGoogle Scholar
  15. Berger M, Grignani G, Giostra A, Ferrari S, Ferraresi V, Tamburini A, Cefalo G, Carnevale-Schianca F, Vassallo E, Picci P, Pagano M, Aglietta M, Pellerito RE, Fagioli F (2012) 153Samarium-EDTMP administration followed by hematopoietic stem cell support for bone metastases in osteosarcoma patients. Ann Oncol 23:1899–1905PubMedGoogle Scholar
  16. Bertario L, Russo A, Sala P, Eboli M, Giarola M, D’amico F, Gismondi V, Varesco L, Pierotti MA, Radice P (2001) Genotype and phenotype factors as determinants of desmoid tumors in patients with familial adenomatous polyposis. Int J Cancer 95:102–107PubMedGoogle Scholar
  17. Bhatia S, Krailo MD, Chen Z, Burden L, Askin FB, Dickman PS, Grier HE, Link MP, Meyers PA, Perlman EJ, Rausen AR, Robison LL, Vietti TJ, Miser JS (2007) Therapy-related myelodysplasia and acute myeloid leukemia after Ewing sarcoma and primitive neuroectodermal tumor of bone: a report from the Children’s Oncology Group. Blood 109:46–51PubMedPubMedCentralGoogle Scholar
  18. Bhatia S, Constine LS (2010) Secondary primary cancers (Chapter 20). In: Halperin EC, Constine LC, Tarbell NJ, Kun LE (eds) Pediatric radiation oncology. Lippincott Williams & Wilkins, Philadelphia, PAGoogle Scholar
  19. Bielack SS, Kempf-Bielack B, Delling G, Exner GU, Flege S, Helmke K, Kotz R, Salzer-Kuntschik M, Werner M, Winkelmann W, Zoubek A, Jurgens H, Winkler K (2002) Prognostic factors in high-grade osteosarcoma of the extremities or trunk: an analysis of 1,702 patients treated on neoadjuvant cooperative osteosarcoma study group protocols. J Clin Oncol 20:776–790PubMedGoogle Scholar
  20. Bisogno G, Ferrari A, Bergeron C, Scagnellato A, Prete A, Alaggio R, Casanova M, D’angelo P, Di Cataldo A, Carli M (2005) The IVADo regimen--a pilot study with ifosfamide, vincristine, actinomycin D, and doxorubicin in children with metastatic soft tissue sarcoma: a pilot study of behalf of the European pediatric Soft tissue sarcoma Study Group. Cancer 103:1719–1724PubMedGoogle Scholar
  21. Bisogno G, Tagarelli A, Stramare R, Beltrame V, Carli M (2013) Hydroxyurea treatment can avoid the need for aggressive surgery in pediatric fibromatosis. J Pediatr Hematol Oncol 35:e171–e173PubMedGoogle Scholar
  22. Blackmon SH, Shah N, Roth JA, Correa AM, Vaporciyan AA, Rice DC, Hofstetter W, Walsh GL, Benjamin R, Pollock R, Swisher SG, Mehran R (2009) Resection of pulmonary and extrapulmonary sarcomatous metastases is associated with long-term survival. Ann Thorac Surg 88:877–884; discussion 884–5PubMedGoogle Scholar
  23. Blakely ML, Spurbeck WW, Pappo AS, Pratt CB, Rodriguez-Galindo C, Santana VM, Merchant TE, Prichard M, Rao BN (1999) The impact of margin of resection on outcome in pediatric nonrhabdomyosarcoma soft tissue sarcoma. J Pediatr Surg 34:672–675PubMedGoogle Scholar
  24. Bossi A, De Wever I, Van Limbergen E, Vanstraelen B (2007) Intensity modulated radiation-therapy for preoperative posterior abdominal wall irradiation of retroperitoneal liposarcomas. Int J Radiat Oncol Biol Phys 67:164–170PubMedGoogle Scholar
  25. Bramwell V, Rouesse J, Steward W, Santoro A, Schraffordt-Koops H, Buesa J, Ruka W, Priario J, Wagener T, Burgers M et al (1994) Adjuvant CYVADIC chemotherapy for adult soft tissue sarcoma—reduced local recurrence but no improvement in survival: a study of the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group. J Clin Oncol 12:1137–1149PubMedGoogle Scholar
  26. Brasme JF, Morfouace M, Grill J, Martinot A, Amalberti R, Bons-Letouzey C, Chalumeau M (2012) Delays in diagnosis of paediatric cancers: a systematic review and comparison with expert testimony in lawsuits. Lancet Oncol 13:e445–e459PubMedGoogle Scholar
  27. Brennan B, De Salvo GL, Orbach D, De Paoli A, Kelsey A, Mudry P, Francotte N, Van Noesel M, Bisogno G, Casanova M, Ferrari A (2016) Outcome of extracranial malignant rhabdoid tumours in children registered in the European Paediatric Soft Tissue Sarcoma Study Group Non-Rhabdomyosarcoma Soft Tissue Sarcoma 2005 Study-EpSSG NRSTS 2005. Eur J Cancer 60:69–82PubMedGoogle Scholar
  28. Brouns F, Stas M, De Wever I (2003) Delay in diagnosis of soft tissue sarcomas. Eur J Surg Oncol 29:440–445PubMedGoogle Scholar
  29. Brown LC, Lester RA, Grams MP, Haddock MG, Olivier KR, Arndt CA, Rose PS, Laack NN (2014) Stereotactic body radiotherapy for metastatic and recurrent ewing sarcoma and osteosarcoma. Sarcoma 2014:418270PubMedPubMedCentralGoogle Scholar
  30. Buitendijk S, Van de Ven CP, Dumans TG, Den Hollander JC, Nowak PJ, Tissing WJ, Pieters R, Van Den Heuvel-Eibrink MM (2005) Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature. Cancer 104:1090–1099PubMedGoogle Scholar
  31. Burgers JM, Van Glabbeke M, Busson A, Cohen P, Mazabraud AR, Abbatucci JS, Kalifa C, Tubiana M, Lemerle JS, Voute PA et al (1988) Osteosarcoma of the limbs. Report of the EORTC-SIOP 03 trial 20781 investigating the value of adjuvant treatment with chemotherapy and/or prophylactic lung irradiation. Cancer 61:1024–1031PubMedGoogle Scholar
  32. Cahlon O, Brennan MF, Jia X, Qin LX, Singer S, Alektiar KM (2012) A postoperative nomogram for local recurrence risk in extremity soft tissue sarcomas after limb-sparing surgery without adjuvant radiation. Ann Surg 255:343–347PubMedPubMedCentralGoogle Scholar
  33. Calvo FA, Sole CV, Cambeiro M, Montero A, Polo A, Gonzalez C, Cuervo M, San Julian M, Garcia-Sabrido JL, Martinez-Monge R (2014) Prognostic value of external beam radiation therapy in patients treated with surgical resection and intraoperative electron beam radiation therapy for locally recurrent soft tissue sarcoma: a multicentric long-term outcome analysis. Int J Radiat Oncol Biol Phys 88:143–150PubMedGoogle Scholar
  34. Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, Cecchetto G, Alaggio R, De Sio L, Koscielniak E, Sotti G, Treuner J (2005) Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group. J Clin Oncol 23:8422–8430PubMedGoogle Scholar
  35. Casali PG, Blay JY (2010) Soft tissue sarcomas: ESMO Clinical Practice guidelines for diagnosis, treatment and follow-up. Ann Oncol 21(Suppl 5):v198–v203PubMedGoogle Scholar
  36. Casson AG, Putnam JB, Natarajan G, Johnston DA, Mountain C, Mcmurtrey M, Roth JA (1992) Five-year survival after pulmonary metastasectomy for adult soft tissue sarcoma. Cancer 69:662–668PubMedGoogle Scholar
  37. Catton C, Davis A, Bell R, O’sullivan B, Fornasier V, Wunder J, Mclean M (1996) Soft tissue sarcoma of the extremity. Limb salvage after failure of combined conservative therapy. Radiother Oncol 41:209–214PubMedGoogle Scholar
  38. Cecchetto G, Guglielmi M, Inserra A, Zanetti I, Dall’igna P, Gigante C, Carli M (2001) Primary re-excision: the Italian experience in patients with localized soft-tissue sarcomas. Pediatr Surg Int 17:532–534PubMedGoogle Scholar
  39. Chang F, Syrjanen S, Syrjanen K (1995) Implications of the p53 tumor-suppressor gene in clinical oncology. J Clin Oncol 13:1009–1022PubMedGoogle Scholar
  40. Chen YW, Huang MY, Chang CC, Lee CS, Liao YM, Chiu SS, Chang TT (2007) FDG PET/CT findings of epithelioid sarcoma in a pediatric patient. Clin Nucl Med 32:898–901PubMedGoogle Scholar
  41. Cheng H, Miura JT, Lalehzari M, Rajeev R, Donahue AE, Bedi M, Gamblin TC, Turaga KK, Johnston FM (2016) Neoadjuvant radiotherapy for retroperitoneal sarcoma: a systematic review. J Surg Oncol 113:628–634PubMedGoogle Scholar
  42. Chui CH, Spunt SL, Liu T, Pappo AS, Davidoff AM, Rao BN, Shochat SJ (2002) Is reexcision in pediatric nonrhabdomyosarcoma soft tissue sarcoma necessary after an initial unplanned resection? J Pediatr Surg 37:1424–1429PubMedGoogle Scholar
  43. Church JM (1998) Mucosal ischemia caused by desmoid tumors in patients with familial adenomatous polyposis: report of four cases. Dis Colon Rectum 41:661–663PubMedGoogle Scholar
  44. Coffin CM, Dehner LP (1986) Soft tissue neoplasms in children: a clinico-pathologic overview. In: Finegold M (ed) Pathology of Neoplasia in children and adolescents. WB Saunders, Philadelphia, PAGoogle Scholar
  45. Coindre JM, Terrier P, Bui NB, Bonichon F, Collin F, Le Doussal V, Mandard AM, Vilain MO, Jacquemier J, Duplay H, Sastre X, Barlier C, Henry-Amar M, Mace-Lesech J, Contesso G (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
  46. Coindre JM, Terrier P, Guillou L, Le Doussal V, Collin F, Ranchere D, Sastre X, Vilain MO, Bonichon F, N’Guyen Bui B (2001) Predictive value of grade for metastasis development in the main histologic types of adult soft tissue sarcomas: a study of 1240 patients from the French Federation of Cancer Centers Sarcoma Group. Cancer 91:1914–1926PubMedGoogle Scholar
  47. Colvin OM (1999) An overview of cyclophosphamide development and clinical applications. Curr Pharm Des 5:555–560PubMedGoogle Scholar
  48. Costa J, Wesley RA, Glatstein E, Rosenberg SA (1984) The grading of soft tissue sarcomas. Results of a clinicohistopathologic correlation in a series of 163 cases. Cancer 53:530–541PubMedGoogle Scholar
  49. Daigeler A, Kuhnen C, Moritz R, Stricker I, Goertz O, Tilkorn D, Steinstraesser L, Steinau HU, Lehnhardt M (2009) Lymph node metastases in soft tissue sarcomas: a single center analysis of 1,597 patients. Langenbeck’s Arch Surg 394:321–329Google Scholar
  50. Dall’Igna P, De Corti F, Alaggio R, Cecchetto G (2014) Sentinel node biopsy in pediatric patients: the experience in a single institution. Eur J Pediatr Surg 24:482–487PubMedGoogle Scholar
  51. Dantonello TM, Int-Veen C, Harms D, Leuschner I, Schmidt BF, Herbst M, Juergens H, Scheel-Walter HG, Bielack SS, Klingebiel T, Dickerhoff R, Kirsch S, Brecht I, Schmelzle R, Greulich M, Gadner H, Greiner J, Marky I, Treuner J, Koscielniak E (2009) Cooperative trial CWS-91 for localized soft tissue sarcoma in children, adolescents, and young adults. J Clin Oncol 27:1446–1455PubMedGoogle Scholar
  52. 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 20:4472–4477PubMedGoogle Scholar
  53. Davis AM, O’sullivan B, Turcotte R, Bell R, Catton C, Chabot P, Wunder J, Hammond A, Benk V, Kandel R, Goddard K, Freeman C, Sadura A, Zee B, Day A, Tu D, Pater J (2005) Late radiation morbidity following randomization to preoperative versus postoperative radiotherapy in extremity soft tissue sarcoma. Radiother Oncol 75:48–53PubMedGoogle Scholar
  54. De Cian F, Delay E, Rudigoz RC, Ranchere D, Rivoire M (1999) Desmoid tumor arising in a cesarean section scar during pregnancy: monitoring and management. Gynecol Oncol 75:145–148PubMedGoogle Scholar
  55. Decou JM, Rao BN, Parham DM, Lobe TE, Bowman L, Pappo AS, Fontanesi J (1995) Malignant peripheral nerve sheath tumors: the St. Jude Children’s Research Hospital experience. Ann Surg Oncol 2:524–529PubMedGoogle Scholar
  56. Delaney TF, Park L, Goldberg SI, Hug EB, Liebsch NJ, Munzenrider JE, Suit HD (2005) Radiotherapy for local control of osteosarcoma. Int J Radiat Oncol Biol Phys 61:492–498PubMedGoogle Scholar
  57. Demetri GD, Von Mehren M, Jones RL, Hensley ML, Schuetze SM, Staddon A, Milhem M, Elias A, Ganjoo K, Tawbi H, Van Tine BA, Spira A, Dean A, Khokhar NZ, Park YC, Knoblauch RE, Parekh TV, Maki RG, Patel SR (2016) Efficacy and Safety of Trabectedin or Dacarbazine for Metastatic Liposarcoma or Leiomyosarcoma After Failure of Conventional Chemotherapy: Results of a Phase III Randomized Multicenter Clinical Trial. J Clin Oncol 34:786–793PubMedGoogle Scholar
  58. Demicco EG, Maki RG, Lev DC, Lazar AJ (2012) New therapeutic targets in soft tissue sarcoma. Adv Anat Pathol 19:170–180PubMedPubMedCentralGoogle Scholar
  59. Dhakal S, Corbin KS, Milano MT, Philip A, Sahasrabudhe D, Jones C, Constine LS (2012) Stereotactic body radiotherapy for pulmonary metastases from soft-tissue sarcomas: excellent local lesion control and improved patient survival. Int J Radiat Oncol Biol Phys 82:940–945PubMedGoogle Scholar
  60. Dickie CI, Parent AL, Griffin AM, Fung S, Chung PW, Catton CN, Ferguson PC, Wunder JS, Bell RS, Sharpe MB, O’sullivan B (2009) Bone fractures following external beam radiotherapy and limb-preservation surgery for lower extremity soft tissue sarcoma: relationship to irradiated bone length, volume, tumor location and dose. Int J Radiat Oncol Biol Phys 75:1119–1124PubMedGoogle Scholar
  61. Dillon PW, Whalen TV, Azizkhan RG, Haase GM, Coran AG, King DR, Smith M (1995) Neonatal soft tissue sarcomas: the influence of pathology on treatment and survival. Children’s Cancer Group Surgical Committee. J Pediatr Surg 30:1038–1041PubMedGoogle Scholar
  62. Donaldson SS (1993) Lessons from our children. Int J Radiat Oncol Biol Phys 26:739–749PubMedGoogle Scholar
  63. Donaldson SS, Torrey M, Link MP, Glicksman A, Gilula L, Laurie F, Manning J, Neff J, Reinus W, Thompson E, Shuster JJ (1998) A multidisciplinary study investigating radiotherapy in Ewing’s sarcoma: end results of POG #8346. Pediatric Oncology Group. Int J Radiat Oncol Biol Phys 42:125–135PubMedGoogle Scholar
  64. Durr HR, Pellengahr C, Nerlich A, Baur A, Maier M, Jansson V (2004) Stewart-Treves syndrome as a rare complication of a hereditary lymphedema. Vasa 33:42–45PubMedGoogle Scholar
  65. Edge S, Byrd D, Compton C et al (2010) AJCC cancer staging manual. Springer, New YorkGoogle Scholar
  66. Eilber F, Giuliano A, Eckardt J, Patterson K, Moseley S, Goodnight J (1987) Adjuvant chemotherapy for osteosarcoma: a randomized prospective trial. J Clin Oncol 5:21–26PubMedGoogle Scholar
  67. Eilber FC, Brennan MF, Eilber FR, Eckardt JJ, Grobmyer SR, Riedel E, Forscher C, Maki RG, Singer S (2007) Chemotherapy is associated with improved survival in adult patients with primary extremity synovial sarcoma. Ann Surg 246:105–113PubMedPubMedCentralGoogle Scholar
  68. Eilber FC, Rosen G, Eckardt J, Forscher C, Nelson SD, Selch M, Dorey F, Eilber FR (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
  69. Enneking WF (1986) A system of staging musculoskeletal neoplasms. Clin Orthop Relat Res:9–24Google Scholar
  70. Enneking WF, Spanier SS, Goodman MA (1980) A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res:106–120Google Scholar
  71. Espat NJ, Bilsky M, Lewis JJ, Leung D, Brennan MF (2002) Soft tissue sarcoma brain metastases. Prevalence in a cohort of 3829 patients. Cancer 94:2706–2711PubMedGoogle Scholar
  72. Evans DG, Baser ME, Mcgaughran J, Sharif S, Howard E, Moran A (2002) Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet 39:311–314PubMedPubMedCentralGoogle Scholar
  73. Falk H, Herbert J, Crowley S, Ishak KG, Thomas LB, Popper H, Caldwell GG (1981) Epidemiology of hepatic angiosarcoma in the United States: 1964-1974. Environ Health Perspect 41:107–113PubMedPubMedCentralGoogle Scholar
  74. Faulkner LB, Hajdu SI, Kher U, La Quaglia M, Exelby PR, Heller G, Wollner N (1995) Pediatric desmoid tumor: retrospective analysis of 63 cases. J Clin Oncol 13:2813–2818PubMedGoogle Scholar
  75. Fernandez-Pineda I, Hudson MM, Pappo AS, Bishop MW, Klosky JL, Brinkman TM, Srivastava DK, Neel MD, Rao BN, Davidoff AM, Krull KR, Mulrooney DA, Robison LL, Ness KK (2016) Long-term functional outcomes and quality of life in adult survivors of childhood extremity sarcomas: a report from the St. Jude Lifetime Cohort Study. J Cancer Surviv 11(1):1–12PubMedPubMedCentralGoogle Scholar
  76. Ferner RE, Golding JF, Smith M, Calonje E, Jan W, Sanjayanathan V, O’Doherty M (2008) [18F]2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) as a diagnostic tool for neurofibromatosis 1 (NF1) associated malignant peripheral nerve sheath tumours (MPNSTs): a long-term clinical study. Ann Oncol 19:390–394PubMedGoogle Scholar
  77. Ferrari A, Casanova M, Collini P, Meazza C, Luksch R, Massimino M, Cefalo G, Terenziani M, Spreafico F, Catania S, Gandola L, Gronchi A, Mariani L, Fossati-Bellani F (2005) Adult-type soft tissue sarcomas in pediatric-age patients: experience at the Istituto Nazionale Tumori in Milan. J Clin Oncol 23:4021–4030PubMedGoogle Scholar
  78. Ferrari A, De Salvo GL, Brennan B, Van Noesel MM, De Paoli A, Casanova M, Francotte N, Kelsey A, Alaggio R, Oberlin O, Carli M, Ben-Arush M, Bergeron C, Merks JH, Jenney M, Stevens MC, Bisogno G, Orbach D (2015) Synovial sarcoma in children and adolescents: the European Pediatric Soft Tissue Sarcoma Study Group prospective trial (EpSSG NRSTS 2005). Ann Oncol 26:567–572PubMedGoogle Scholar
  79. Ferrari A, Gronchi A, Casanova M, Meazza C, Gandola L, Collini P, Lozza L, Bertulli R, Olmi P, Casali PG (2004) Synovial sarcoma: a retrospective analysis of 271 patients of all ages treated at a single institution. Cancer 101:627–634PubMedGoogle Scholar
  80. Ferrari A, Miceli R, Meazza C, Zaffignani E, Gronchi A, Piva L, Collini P, Podda M, Massimino M, Luksch R, Cefalo G, Terenziani M, Spreafico F, Polastri D, Fossati-Bellani F, Casanova M, Mariani L (2009) Soft tissue sarcomas of childhood and adolescence: the prognostic role of tumor size in relation to patient body size. J Clin Oncol 27:371–376PubMedGoogle Scholar
  81. Ferrari A, Miceli R, Rey A, Oberlin O, Orbach D, Brennan B, Mariani L, Carli M, Bisogno G, Cecchetto G, De Salvo GL, Casanova M, Vannoesel MM, Kelsey A, Stevens MC, Devidas M, Pappo AS, Spunt SL (2011a) Non-metastatic unresected paediatric non-rhabdomyosarcoma soft tissue sarcomas: results of a pooled analysis from United States and European groups. Eur J Cancer 47:724–731PubMedGoogle Scholar
  82. Ferrari A, Sultan I, Huang TT, Rodriguez-Galindo C, Shehadeh A, Meazza C, Ness KK, Casanova M, Spunt SL (2011b) Soft tissue sarcoma across the age spectrum: a population-based study from the Surveillance Epidemiology and End Results database. Pediatr Blood Cancer 57:943–949PubMedPubMedCentralGoogle Scholar
  83. Flamant F, Rodary C, Rey A, Praquin MT, Sommelet D, Quintana E, Theobald S, Brunat-Mentigny M, Otten J, Voute PA, Habrand JL, Martelli H, Barrett A, Terrier-Lacombe MJ, Oberlin O (1998) Treatment of non-metastatic rhabdomyosarcomas in childhood and adolescence. Results of the second study of the International Society of Paediatric Oncology: MMT84. Eur J Cancer 34:1050–1062PubMedGoogle Scholar
  84. Fleming JB, Cantor SB, Varma DG, Holst D, Feig BW, Hunt KK, Patel SR, Benjamin RS, Pollock RE, Pisters PW (2001) Utility of chest computed tomography for staging in patients with T1 extremity soft tissue sarcomas. Cancer 92:863–868PubMedGoogle Scholar
  85. Fletcher CDM, Bridge JA, Hogendoom PCW et al (2013) WHO classification of tumours of soft tissue and bone, 4th edn. International Agency for Research on Cancer, Lyon, FranceGoogle Scholar
  86. Fogliata A, Scorsetti M, Navarria P, Catalano M, Clivio A, Cozzi L, Lobefalo F, Nicolini G, Palumbo V, Pellegrini C, Reggiori G, Roggio A, Vanetti E, Alongi F, Pentimalli S, Mancosu P (2013) Dosimetric comparison between VMAT with different dose calculation algorithms and protons for soft-tissue sarcoma radiotherapy. Acta Oncol 52:545–552PubMedGoogle Scholar
  87. Folkert MR, Singer S, Brennan MF, Kuk D, Qin LX, Kobayashi WK, Crago AM, Alektiar KM (2014a) Comparison of local recurrence with conventional and intensity-modulated radiation therapy for primary soft-tissue sarcomas of the extremity. J Clin Oncol 32:3236–3241PubMedPubMedCentralGoogle Scholar
  88. Folkert MR, Tong WY, Laquaglia MP, Wexler LH, Chou AJ, Magnan H, Zelefsky MJ, Wolden SL (2014b) 20-year experience with intraoperative high-dose-rate brachytherapy for pediatric sarcoma: outcomes, toxicity, and practice recommendations. Int J Radiat Oncol Biol Phys 90:362–368PubMedGoogle Scholar
  89. Fong Y, Coit DG, Woodruff JM, Brennan MF (1993) Lymph node metastasis from soft tissue sarcoma in adults. Analysis of data from a prospective database of 1772 sarcoma patients. Ann Surg 217:72–77PubMedPubMedCentralGoogle Scholar
  90. Friedmann D, Wunder JS, Ferguson P, O’sullivan B, Roberge D, Catton C, Freeman C, Saran N, Turcotte RE (2011) Incidence and Severity of Lymphoedema following Limb Salvage of Extremity Soft Tissue Sarcoma. Sarcoma 2011:289673PubMedPubMedCentralGoogle Scholar
  91. Friend SH, Bernards R, Rogelj S, Weinberg RA, Rapaport JM, Albert DM, Dryja TP (1986) A human DNA segment with properties of the gene that predisposes to retinoblastoma and osteosarcoma. Nature 323:643–646PubMedGoogle Scholar
  92. Gadd S, Beezhold P, Jennings L, George D, Leuer K, Huang CC, Huff V, Tognon C, Sorensen PH, Triche T, Coffin CM, Perlman EJ (2012) Mediators of receptor tyrosine kinase activation in infantile fibrosarcoma: a Children’s Oncology Group study. J Pathol 228:119–130PubMedGoogle Scholar
  93. Gansar GF, Markowitz IP, Cerise EJ (1987) Thirty years of experience with desmoid tumors at Charity Hospital. Am Surg 53:318–319PubMedGoogle Scholar
  94. Garcia-Carbonero R, Supko JG, Manola J, Seiden MV, Harmon D, Ryan DP, Quigley MT, Merriam P, Canniff J, Goss G, Matulonis U, Maki RG, Lopez T, Puchalski TA, Sancho MA, Gomez J, Guzman C, Jimeno J, Demetri GD (2004) Phase II and pharmacokinetic study of ecteinascidin 743 in patients with progressive sarcomas of soft tissues refractory to chemotherapy. J Clin Oncol 22:1480–1490PubMedGoogle Scholar
  95. Gawade PL, Hudson MM, Kaste SC, Neglia JP, Wasilewski-Masker K, Constine LS, Robison LL, Ness KK (2014) A systematic review of selected musculoskeletal late effects in survivors of childhood cancer. Curr Pediatr Rev 10:249–262PubMedGoogle Scholar
  96. Gega M, Yanagi H, Yoshikawa R, Noda M, Ikeuchi H, Tsukamoto K, Oshima T, Fujiwara Y, Gondo N, Tamura K, Utsunomiya J, Hashimoto-Tamaoki T, Yamamura T (2006) Successful chemotherapeutic modality of doxorubicin plus dacarbazine for the treatment of desmoid tumors in association with familial adenomatous polyposis. J Clin Oncol 24:102–105PubMedGoogle Scholar
  97. Girard P, Spaggiari L, Baldeyrou P, Le Chevalier T, Le Cesne A, Escudier B, Filaire M, Grunenwald D (1997) Should the number of pulmonary metastases influence the surgical decision? Eur J Cardiothorac Surg 12:385–391; discussion 392PubMedGoogle Scholar
  98. Gitelis S, Thomas R, Templeton A, Schajowicz F (1989) Characterization of the pseudocapsule of soft-tissue sarcomas. An experimental study in rats. Clin Orthop Relat Res:285–292Google Scholar
  99. Giuliano AE, Eilber FR (1985) The rationale for planned reoperation after unplanned total excision of soft-tissue sarcomas. J Clin Oncol 3:1344–1348PubMedGoogle Scholar
  100. Goldblum JR, Fletcher JA (2002) Desmoid-type fibromatosis. In: Fletcher CD, Unnl KK, Mertens F (eds) World Health Organization classification of tumours: pathology and genetics of tumours of soft tissue and bone. International Agency for Reserach on Cancer Press, Lyon, FranceGoogle Scholar
  101. Gomez Garcia EB, Knoers NV (2009) Gardner’s syndrome (familial adenomatous polyposis): a cilia-related disorder. Lancet Oncol 10:727–735PubMedGoogle Scholar
  102. Goto M, Miller RW, Ishikawa Y, Sugano H (1996) Excess of rare cancers in Werner syndrome (adult progeria). Cancer Epidemiol Biomark Prev 5:239–246Google Scholar
  103. Greenberg HM, Goebel R, Weichselbaum RR, Greenberger JS, Chaffey JT, Cassady JR (1981) Radiation therapy in the treatment of aggressive fibromatoses. Int J Radiat Oncol Biol Phys 7:305–310PubMedGoogle Scholar
  104. Groen EJ, Roos A, Muntinghe FL, Enting RH, De Vries J, Kleibeuker JH, Witjes MJ, Links TP, Van Beek AP (2008) Extra-intestinal manifestations of familial adenomatous polyposis. Ann Surg Oncol 15:2439–2450PubMedPubMedCentralGoogle Scholar
  105. Guadagnolo BA, Zagars GK, Ballo MT (2008) Long-term outcomes for desmoid tumors treated with radiation therapy. Int J Radiat Oncol Biol Phys 71:441–447PubMedGoogle Scholar
  106. Guillou L, Coindre JM, Bonichon F, Nguyen BB, Terrier P, Collin F, Vilain MO, Mandard AM, Le Doussal V, Leroux A, Jacquemier J, Duplay H, Sastre-Garau X, Costa J (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 15:350–362PubMedGoogle Scholar
  107. Gutierrez JC, Perez EA, Moffat FL, Livingstone AS, Franceschi D, Koniaris LG (2007) Should soft tissue sarcomas be treated at high-volume centers? An analysis of 4205 patients. Ann Surg 245:952–958PubMedPubMedCentralGoogle Scholar
  108. Gutman M, Inbar M, Lev-Shlush D, Abu-Abid S, Mozes M, Chaitchik S, Meller I, Klausner JM (1997) High dose tumor necrosis factor-alpha and melphalan administered via isolated limb perfusion for advanced limb soft tissue sarcoma results in a >90% response rate and limb preservation. Cancer 79:1129–1137PubMedGoogle Scholar
  109. Haimi M, Peretz Nahum M, Ben Arush MW (2004) Delay in diagnosis of children with cancer: a retrospective study of 315 children. Pediatr Hematol Oncol 21:37–48PubMedGoogle Scholar
  110. Hansmann A, Adolph C, Vogel T, Unger A, Moeslein G (2004) High-dose tamoxifen and sulindac as first-line treatment for desmoid tumors. Cancer 100:612–620PubMedGoogle Scholar
  111. Hawkins DS, Spunt SL, Skapek SX, Committee COGSTS (2013) Children’s Oncology Group’s 2013 blueprint for research: Soft tissue sarcomas. Pediatr Blood Cancer 60:1001–1008PubMedGoogle Scholar
  112. Hawkins MM (1990) Second primary tumors following radiotherapy for childhood cancer. Int J Radiat Oncol Biol Phys 19:1297–1301PubMedGoogle Scholar
  113. Hayani A, Mahoney DH Jr, Hawkins HK, Steuber CP, Hurwitz R, Fernbach DJ (1992) Soft-tissue sarcomas other than rhabdomyosarcoma in children. Med Pediatr Oncol 20:114–118PubMedGoogle Scholar
  114. Hayes-Jordan A, Green HL, Lin H, Owusu-Agyemang P, Fitzgerald N, Arunkumar R, Mejia R, Okhuysen-Cawley R, Mauricio R, Fournier K, Ludwig J, Anderson P (2014) Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor. Ann Surg Oncol 21:220–224PubMedGoogle Scholar
  115. Hays DM, Lawrence W Jr, Wharam M, Newton W Jr, Ruymann FB, Beltangady M, Maurer HM (1989) Primary reexcision for patients with ‘microscopic residual’ tumor following initial excision of sarcomas of trunk and extremity sites. J Pediatr Surg 24:5–10PubMedGoogle Scholar
  116. Hegazy MA, Kotb SZ, Sakr H, El Dosoky E, Amer T, Hegazi RA, Farouk O (2007) Preoperative isolated limb infusion of Doxorubicin and external irradiation for limb-threatening soft tissue sarcomas. Ann Surg Oncol 14:568–576PubMedGoogle Scholar
  117. Heinrich MC, Joensuu H, Demetri GD, Corless CL, Apperley J, Fletcher JA, Soulieres D, Dirnhofer S, Harlow A, Town A, Mckinley A, Supple SG, Seymour J, Di Scala L, Van Oosterom A, Herrmann R, Nikolova Z, Mcarthur AG (2008) Phase Ii, open-label study evaluating the activity of imatinib in treating life-threatening malignancies known to be associated with imatinib-sensitive tyrosine kinases. Clin Cancer Res 14:2717–2725PubMedGoogle Scholar
  118. Heinrich MC, Mcarthur GA, Demetri GD, Joensuu H, Bono P, Herrmann R, Hirte H, Cresta S, Koslin DB, Corless CL, Dirnhofer S, Van Oosterom AT, Nikolova Z, Dimitrijevic S, Fletcher JA (2006) Clinical and molecular studies of the effect of imatinib on advanced aggressive fibromatosis (desmoid tumor). J Clin Oncol 24:1195–1203PubMedGoogle Scholar
  119. Henderson TO, Rajaraman P, Stovall M, Constine LS, Olive A, Smith SA, Mertens A, Meadows A, Neglia JP, Hammond S, Whitton J, Inskip PD, Robison LL, Diller L (2012) Risk factors associated with secondary sarcomas in childhood cancer survivors: a report from the childhood cancer survivor study. Int J Radiat Oncol Biol Phys 84:224–230PubMedPubMedCentralGoogle Scholar
  120. Henderson TO, Whitton J, Stovall M, Mertens AC, Mitby P, Friedman D, Strong LC, Hammond S, Neglia JP, Meadows AT, Robison L, Diller L (2007) Secondary sarcomas in childhood cancer survivors: a report from the Childhood Cancer Survivor Study. J Natl Cancer Inst 99:300–308PubMedGoogle Scholar
  121. Hingorani P, Janeway K, Crompton BD, Kadoch C, Mackall CL, Khan J, Shern JF, Schiffman J, Mirabello L, Savage SA, Ladanyi M, Meltzer P, Bult CJ, Adamson PC, Lupo PJ, Mody R, Dubois SG, Parsons DW, Khanna C, Lau C, Hawkins DS, Randall RL, Smith M, Sorensen PH, Plon SE, Skapek SX, Lessnick S, Gorlick R, Reed DR (2016) Current state of pediatric sarcoma biology and opportunities for future discovery: A report from the sarcoma translational research workshop. Cancer Genet 209:182–194PubMedPubMedCentralGoogle Scholar
  122. Hong DS, Garrido-Laguna I, Ekmekcioglu S, Falchook GS, Naing A, Wheler JJ, Fu S, Moulder SL, Piha-Paul S, Tsimberidou AM, Wen Y, Culotta KS, Anderes K, Davis DW, Liu W, George GC, Camacho LH, Percy Ivy S, Kurzrock R (2014) Dual inhibition of the vascular endothelial growth factor pathway: a phase 1 trial evaluating bevacizumab and AZD2171 (cediranib) in patients with advanced solid tumors. Cancer 120:2164–2173PubMedGoogle Scholar
  123. Horowitz ME, Pratt CB, Webber BL, Hustu HO, Etcubanas E, Miliauskas J, Rao BN, Fleming ID, Mahesh Kumar AP, Green AA (1986) Therapy for childhood soft-tissue sarcomas other than rhabdomyosarcoma: a review of 62 cases treated at a single institution. J Clin Oncol 4:559–564PubMedGoogle Scholar
  124. Hudson MM, Mertens AC, Yasui Y, Hobbie W, Chen H, Gurney JG, Yeazel M, Recklitis CJ, Marina N, Robison LR, Oeffinger KC (2003) Health status of adult long-term survivors of childhood cancer: a report from the Childhood Cancer Survivor Study. JAMA 290:1583–1592PubMedGoogle Scholar
  125. Indelicato DJ, Meadows K, Gibbs CP Jr, Morris CG, Scarborough MT, Zlotecki RA (2009) Effectiveness and morbidity associated with reirradiation in conservative salvage management of recurrent soft-tissue sarcoma. Int J Radiat Oncol Biol Phys 73:267–272PubMedGoogle Scholar
  126. Inskip PD, Sigurdson AJ, Veiga L, Bhatti P, Ronckers C, Rajaraman P, Boukheris H, Stovall M, Smith S, Hammond S, Henderson TO, Watt TC, Mertens AC, Leisenring W, Stratton K, Whitton J, Donaldson SS, Armstrong GT, Robison LL, Neglia JP (2016) Radiation-related new primary solid cancers in the childhood cancer survivor study: comparative radiation dose response and modification of treatment effects. Int J Radiat Oncol Biol Phys 94:800–807PubMedGoogle Scholar
  127. Issels RD, Lindner LH, Verweij J, Wust P, Reichardt P, Schem BC, Abdel-Rahman S, Daugaard S, Salat C, Wendtner CM, Vujaskovic Z, Wessalowski R, Jauch KW, Durr HR, Ploner F, Baur-Melnyk A, Mansmann U, Hiddemann W, Blay JY, Hohenberger P (2010) Neo-adjuvant chemotherapy alone or with regional hyperthermia for localised high-risk soft-tissue sarcoma: a randomised phase 3 multicentre study. Lancet Oncol 11:561–570PubMedPubMedCentralGoogle Scholar
  128. Italiano A (2012) PAZOPANIB Efficacy and Tolerance in Desmoids Tumors (DESMOPAZ). Identifier: NCT01876082
  129. Jablons D, Steinberg SM, Roth J, Pittaluga S, Rosenberg SA, Pass HI (1989) Metastasectomy for soft tissue sarcoma. Further evidence for efficacy and prognostic indicators. J Thorac Cardiovasc Surg 97:695–705PubMedGoogle Scholar
  130. Jaffe N, Toth BB, Hoar RE, Ried HL, Sullivan MP, Mcneese MD (1984) Dental and maxillofacial abnormalities in long-term survivors of childhood cancer: effects of treatment with chemotherapy and radiation to the head and neck. Pediatrics 73:816–823PubMedGoogle Scholar
  131. Jager PL, Hoekstra HJ, Leeuw J, Van der Graaf WT, De Vries EG, Piers D (2000) Routine bone scintigraphy in primary staging of soft tissue sarcoma; Is it worthwhile? Cancer 89:1726–1731PubMedGoogle Scholar
  132. Janeway KA, Maki RG (2012) New strategies in sarcoma therapy: linking biology and novel agents. Clin Cancer Res 18:5837–5844PubMedGoogle Scholar
  133. Jingu K, Tsujii H, Mizoe JE, Hasegawa A, Bessho H, Takagi R, Morikawa T, Tonogi M, Tsuji H, Kamada T, Yamada S (2012) Carbon ion radiation therapy improves the prognosis of unresectable adult bone and soft-tissue sarcoma of the head and neck. Int J Radiat Oncol Biol Phys 82:2125–2131PubMedGoogle Scholar
  134. Judson I, Verweij J, Gelderblom H, Hartmann JT, Schoffski P, Blay JY, Kerst JM, Sufliarsky J, Whelan J, Hohenberger P, Krarup-Hansen A, Alcindor T, Marreaud S, Litiere S, Hermans C, Fisher C, Hogendoorn PC, Dei Tos AP, Van Der Graaf WT (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 15:415–423PubMedGoogle Scholar
  135. Kamada T, Tsujii H, Tsuji H, Yanagi T, Mizoe JE, Miyamoto T, Kato H, Yamada S, Morita S, Yoshikawa K, Kandatsu S, Tateishi A (2002) Efficacy and safety of carbon ion radiotherapy in bone and soft tissue sarcomas. J Clin Oncol 20:4466–4471PubMedGoogle Scholar
  136. Kandel R, Coakley N, Werier J, Engel J, Ghert M, Verma S (2013) Surgical margins and handling of soft-tissue sarcoma in extremities: a clinical practice guideline. Curr Oncol 20:e247–e254PubMedPubMedCentralGoogle Scholar
  137. Karavasilis V, Seddon BM, Ashley S, Al-Muderis O, Fisher C, Judson I (2008) Significant clinical benefit of first-line palliative chemotherapy in advanced soft-tissue sarcoma: retrospective analysis and identification of prognostic factors in 488 patients. Cancer 112:1585–1591PubMedGoogle Scholar
  138. Kasper B, Ouali M, Van Glabbeke M, Blay JY, Bramwell VH, Woll PJ, Hohenberger P, Schoffski P (2013) Prognostic factors in adolescents and young adults (AYA) with high risk soft tissue sarcoma (STS) treated by adjuvant chemotherapy: a study based on pooled European Organisation for Research and Treatment of Cancer (EORTC) clinical trials 62771 and 62931. Eur J Cancer 49:449–456PubMedGoogle Scholar
  139. Kaste SC, Ahn H, Liu T, Liu W, Krasin MJ, Hudson MM, Spunt SL (2008) Bone mineral density deficits in pediatric patients treated for sarcoma. Pediatr Blood Cancer 50:1032–1038PubMedGoogle Scholar
  140. Kattan MW, Leung DH, Brennan MF (2002) Postoperative nomogram for 12-year sarcoma-specific death. J Clin Oncol 20:791–796PubMedGoogle Scholar
  141. Kayton ML, Delgado R, Busam K, Cody HS 3rd, Athanasian EA, Coit D, La Quaglia MP (2008) Experience with 31 sentinel lymph node biopsies for sarcomas and carcinomas in pediatric patients. Cancer 112:2052–2059PubMedGoogle Scholar
  142. Kayton ML, Meyers P, Wexler LH, Gerald WL, Laquaglia MP (2006) Clinical presentation, treatment, and outcome of alveolar soft part sarcoma in children, adolescents, and young adults. J Pediatr Surg 41:187–193PubMedGoogle Scholar
  143. Kempf-Bielack B, Bielack SS, Jurgens H, Branscheid D, Berdel WE, Exner GU, Gobel U, Helmke K, Jundt G, Kabisch H, Kevric M, Klingebiel T, Kotz R, Maas R, Schwarz R, Semik M, Treuner J, Zoubek A, Winkler K (2005) Osteosarcoma relapse after combined modality therapy: an analysis of unselected patients in the Cooperative Osteosarcoma Study Group (COSS). J Clin Oncol 23:559–568PubMedGoogle Scholar
  144. Kenney LB, Laufer MR, Grant FD, Grier H, Diller L (2001) High risk of infertility and long term gonadal damage in males treated with high dose cyclophosphamide for sarcoma during childhood. Cancer 91:613–621PubMedGoogle Scholar
  145. Kepka L, Delaney TF, Suit HD, Goldberg SI (2005) Results of radiation therapy for unresected soft-tissue sarcomas. Int J Radiat Oncol Biol Phys 63:852–859PubMedGoogle Scholar
  146. Keus RB, Nout RA, Blay JY, De Jong JM, Hennig I, Saran F, Hartmann JT, Sunyach MP, Gwyther SJ, Ouali M, Kirkpatrick A, Poortmans PM, Hogendoorn PC, Van der Graaf WT (2013) Results of a phase II pilot study of moderate dose radiotherapy for inoperable desmoid-type fibromatosis--an EORTC STBSG and ROG study (EORTC 62991-22998). Ann Oncol 24:2672–2676PubMedGoogle Scholar
  147. Khoja H, Griffin A, Dickson B, Wunder J, Ferguson P, Howarth D, Kandel R (2013) Sampling modality influences the predictive value of grading in adult soft tissue extremity sarcomas. Arch Pathol Lab Med 137:1774–1779PubMedGoogle Scholar
  148. Khoury JD, Coffin CM, Spunt SL, Anderson JR, Meyer WH, Parham DM (2010) Grading of nonrhabdomyosarcoma soft tissue sarcoma in children and adolescents: a comparison of parameters used for the Federation Nationale des Centers de Lutte Contre le Cancer and Pediatric Oncology Group Systems. Cancer 116:2266–2274PubMedPubMedCentralGoogle Scholar
  149. Kiel KD, Suit HD (1984) Radiation therapy in the treatment of aggressive fibromatoses (desmoid tumors). Cancer 54:2051–2055PubMedGoogle Scholar
  150. Kim S, Ott HC, Wright CD, Wain JC, Morse C, Gaissert HA, Donahue DM, Mathisen DJ, Lanuti M (2011) Pulmonary resection of metastatic sarcoma: prognostic factors associated with improved outcomes. Ann Thorac Surg 92:1780–1786; discussion 1786–7PubMedGoogle Scholar
  151. Kirova YM, Feuilhade F, Calitchi E, Otmezguine Y, Le Bourgeois JP (1999) Stewart-Treves syndrome after treatment for breast cancer. Breast 8:282–284PubMedGoogle Scholar
  152. 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 99:24–31PubMedGoogle Scholar
  153. Krasin MJ, Davidoff AM, Xiong X, Wu S, Hua CH, Navid F, Rodriguez-Galindo C, Rao BN, Hoth KA, Neel MD, Merchant TE, Kun LE, Spunt SL (2010) Preliminary results from a prospective study using limited margin radiotherapy in pediatric and young adult patients with high-grade nonrhabdomyosarcoma soft-tissue sarcoma. Int J Radiat Oncol Biol Phys 76:874–878PubMedGoogle Scholar
  154. Krasin MJ, Wiese KM, Spunt SL, Hua CH, Daw N, Navid F, Davidoff AM, Mcgregor L, Merchant TE, Kun LE, Mccrarey L, Hoth KA, Yan X, Xiong X (2012) Jaw dysfunction related to pterygoid and masseter muscle dosimetry after radiation therapy in children and young adults with head-and-neck sarcomas. Int J Radiat Oncol Biol Phys 82:355–360PubMedGoogle Scholar
  155. Kraybill WG, Harris J, Spiro IJ, Ettinger DS, Delaney TF, Blum RH, Lucas DR, Harmon DC, Letson GD, Eisenberg B (2006) Phase II study of neoadjuvant chemotherapy and radiation therapy in the management of high-risk, high-grade, soft tissue sarcomas of the extremities and body wall: Radiation Therapy Oncology Group Trial 9514. J Clin Oncol 24:619–625PubMedGoogle Scholar
  156. Kraybill WG, Harris J, Spiro IJ, Ettinger DS, Delaney TF, Blum RH, Lucas DR, Harmon DC, Letson GD, Eisenberg B (2010) Long-term results of a phase 2 study of neoadjuvant chemotherapy and radiotherapy in the management of high-risk, high-grade, soft tissue sarcomas of the extremities and body wall: Radiation Therapy Oncology Group Trial 9514. Cancer 116:4613–4621PubMedPubMedCentralGoogle Scholar
  157. Kumamoto K, Ishida H, Ohsawa T, Ishibashi K, Ushiama M, Yoshida T, Iwama T (2015) Germline and somatic mutations of the APC gene in papillary thyroid carcinoma associated with familial adenomatous polyposis: analysis of three cases and a review of the literature. Oncol Lett 10:2239–2243PubMedPubMedCentralGoogle Scholar
  158. Kuttesch JF Jr, Wexler LH, Marcus RB, Fairclough D, Weaver-Mcclure L, White M, Mao L, Delaney TF, Pratt CB, Horowitz ME, Kun LE (1996) Second malignancies after Ewing’s sarcoma: radiation dose-dependency of secondary sarcomas. J Clin Oncol 14:2818–2825PubMedGoogle Scholar
  159. Ladanyi M, Cha C, Lewis R, Jhanwar SC, Huvos AG, Healey JH (1993) MDM2 gene amplification in metastatic osteosarcoma. Cancer Res 53:16–18PubMedGoogle Scholar
  160. Laramore GE, Griffith JT, Boespflug M, Pelton JG, Griffin T, Griffin BR, Russell KJ, Koh W, Parker RG, Davis LW (1989) Fast neutron radiotherapy for sarcomas of soft tissue, bone, and cartilage. Am J Clin Oncol 12:320–326PubMedGoogle Scholar
  161. Lazar AJ, Tuvin D, Hajibashi S, Habeeb S, Bolshakov S, Mayordomo-Aranda E, Warneke CL, Lopez-Terrada D, Pollock RE, Lev D (2008) Specific mutations in the beta-catenin gene (CTNNB1) correlate with local recurrence in sporadic desmoid tumors. Am J Pathol 173:1518–1527PubMedPubMedCentralGoogle Scholar
  162. Le Cesne A, Judson I, Crowther D, Rodenhuis S, Keizer HJ, Van Hoesel Q, Blay JY, Frisch J, Van Glabbeke M, Hermans C, Van Oosterom A, Tursz T, Verweij J (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
  163. Link M, Grier HE, Donaldson S (1991) Sarcomas of bone. In: Fernbach D, Vietti T (eds) Clinical pediatric oncology. CRC Press, St. Louis, MOGoogle Scholar
  164. Link MP, Goorin AM, Miser AW, Green AA, Pratt CB, Belasco JB, Pritchard J, Malpas JS, Baker AR, Kirkpatrick JA et al (1986) The effect of adjuvant chemotherapy on relapse-free survival in patients with osteosarcoma of the extremity. N Engl J Med 314:1600–1606PubMedGoogle Scholar
  165. Liu J, Ng D, Lee J, Stalley P, Hong A (2016) Chest wall desmoid tumours treated with definitive radiotherapy: a plan comparison of 3D conformal radiotherapy, intensity-modulated radiotherapy and volumetric-modulated arc radiotherapy. Radiat Oncol 11:34PubMedPubMedCentralGoogle Scholar
  166. Loeb D (2013) Combination of External Beam Radiotherapy With 153Sm-EDTMP to Treat High Risk Osteosarcoma. Identifier: NCT01886105
  167. Lorigan P, Verweij J, Papai Z, Rodenhuis S, Le Cesne A, Leahy MG, Radford JA, Van Glabbeke MM, Kirkpatrick A, Hogendoorn PC, Blay JY (2007) Phase III trial of two investigational schedules of ifosfamide compared with standard-dose doxorubicin in advanced or metastatic soft tissue sarcoma: a European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group Study. J Clin Oncol 25:3144–3150PubMedGoogle Scholar
  168. Loya AC, Prayaga AK, Arora A, Sundaram C, Rao IS, Uppin SG, Raju GS, Surath A, Rajappa RS (2007) Lymph node metastasis of soft tissue tumors: a cytomorphologic study. Acta Cytol 51:153–160PubMedGoogle Scholar
  169. Macdermed DM, Miller LL, Peabody TD, Simon MA, Luu HH, Haydon RC, Montag AG, Undevia SD, Connell PP (2010) Primary tumor necrosis predicts distant control in locally advanced soft-tissue sarcomas after preoperative concurrent chemoradiotherapy. Int J Radiat Oncol Biol Phys 76:1147–1153PubMedGoogle Scholar
  170. Machak GN, Tkachev SI, Solovyev YN, Sinyukov PA, Ivanov SM, Kochergina NV, Ryjkov AD, Tepliakov VV, Bokhian BY, Glebovskaya VV (2003) Neoadjuvant chemotherapy and local radiotherapy for high-grade osteosarcoma of the extremities. Mayo Clin Proc 78:147–155PubMedGoogle Scholar
  171. Magnan H, Abramson SJ, Price AP, Grewal RK, Merchant MS, Laquaglia MP, Meyers PA (2013) Positron emission tomography for response assessment in desmoplastic small round cell tumor. J Pediatr Hematol Oncol 35:e190–e193PubMedGoogle Scholar
  172. Maki RG, D’adamo DR, Keohan ML, Saulle M, Schuetze SM, Undevia SD, Livingston MB, Cooney MM, Hensley ML, Mita MM, Takimoto CH, Kraft AS, Elias AD, Brockstein B, Blachere NE, Edgar MA, Schwartz LH, Qin LX, Antonescu CR, Schwartz GK (2009) Phase II study of sorafenib in patients with metastatic or recurrent sarcomas. J Clin Oncol 27:3133–3140PubMedPubMedCentralGoogle Scholar
  173. Maki RG, Wathen JK, Patel SR, Priebat DA, Okuno SH, Samuels B, Fanucchi M, Harmon DC, Schuetze SM, Reinke D, Thall PF, Benjamin RS, Baker LH, Hensley ML (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 [corrected]. J Clin Oncol 25:2755–2763PubMedGoogle Scholar
  174. Marcus KC, Grier HE, Shamberger RC, Gebhardt MC, Perez-Atayde A, Silver B, Tarbell NJ (1997) Childhood soft tissue sarcoma: a 20-year experience. J Pediatr 131:603–607PubMedGoogle Scholar
  175. Marcus RB Jr (1996) Current controversies in pediatric radiation oncology. Orthop Clin North Am 27:551–557PubMedGoogle Scholar
  176. Marino-Enriquez A (2015) Advances in the molecular analysis of soft tissue tumors and clinical implications. Surg Pathol Clin 8:525–537PubMedPubMedCentralGoogle Scholar
  177. Matsunobu A, Imai R, Kamada T, Imaizumi T, Tsuji H, Tsujii H, Shioyama Y, Honda H, Tatezaki S (2012) Impact of carbon ion radiotherapy for unresectable osteosarcoma of the trunk. Cancer 118:4555–4563PubMedGoogle Scholar
  178. Maurer HM, Beltangady M, Gehan EA, Crist W, Hammond D, Hays DM, Heyn R, Lawrence W, Newton W, Ortega J et al (1988) The Intergroup Rhabdomyosarcoma Study-I. A final report. Cancer 61:209–220PubMedGoogle Scholar
  179. Mazeron JJ, Suit HD (1987) Lymph nodes as sites of metastases from sarcomas of soft tissue. Cancer 60:1800–1808PubMedGoogle Scholar
  180. Mcarthur GA, Demetri GD, Van Oosterom A, Heinrich MC, Debiec-Rychter M, Corless CL, Nikolova Z, Dimitrijevic S, Fletcher JA (2005) Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with imatinib: Imatinib Target Exploration Consortium Study B2225. J Clin Oncol 23:866–873PubMedGoogle Scholar
  181. Mccarville MB, Muzzafar S, Kao SC, Coffin CM, Parham DM, Anderson JR, Spunt SL (2014) Imaging features of alveolar soft-part sarcoma: a report from Children’s Oncology Group Study ARST0332. AJR Am J Roentgenol 203:1345–1352PubMedPubMedCentralGoogle Scholar
  182. Mcclain KL, Leach CT, Jenson HB, Joshi VV, Pollock BH, Parmley RT, Dicarlo FJ, Chadwick EG, Murphy SB (1995) Association of Epstein-Barr virus with leiomyosarcomas in children with AIDS. N Engl J Med 332:12–18PubMedGoogle Scholar
  183. Meadows AT, Friedman DL, Neglia JP, Mertens AC, Donaldson SS, Stovall M, Hammond S, Yasui Y, Inskip PD (2009) Second neoplasms in survivors of childhood cancer: findings from the Childhood Cancer Survivor Study cohort. J Clin Oncol 27:2356–2362PubMedPubMedCentralGoogle Scholar
  184. Meistrich ML, Wilson G, Brown BW, Da Cunha MF, Lipshultz LI (1992) Impact of cyclophosphamide on long-term reduction in sperm count in men treated with combination chemotherapy for Ewing and soft tissue sarcomas. Cancer 70:2703–2712PubMedGoogle Scholar
  185. Melis M, Zager JS, Sondak VK (2008) Multimodality management of desmoid tumors: how important is a negative surgical margin? J Surg Oncol 98:594–602PubMedGoogle Scholar
  186. Merchant MS, Bernstein D, Amoako M, Baird K, Fleisher TA, Morre M, Steinberg SM, Sabatino M, Stroncek DF, Venkatasan AM, Wood BJ, Wright M, Zhang H, Mackall CL (2016) Adjuvant Immunotherapy to Improve Outcome in High-Risk Pediatric Sarcomas. Clin Cancer Res 22:3182–3191PubMedGoogle Scholar
  187. Merchant TE, Nguyen D, Walter AW, Pappo AS, Kun LE, Rao BN (2000a) Long-term results with radiation therapy for pediatric desmoid tumors. Int J Radiat Oncol Biol Phys 47:1267–1271PubMedGoogle Scholar
  188. Merchant TE, Parsh N, Del Valle PL, Coffey DH, Galindo CR, Jenkins JJ, Pappo A, Neel MD, Rao BN (2000b) Brachytherapy for pediatric soft-tissue sarcoma. Int J Radiat Oncol Biol Phys 46:427–432PubMedGoogle Scholar
  189. Mertens F, Antonescu CR, Hohenberger P, Ladanyi M, Modena P, D’incalci M, Casali PG, Aglietta M, Alvegard T (2009) Translocation-related sarcomas Semin Oncol 36:312–323PubMedGoogle Scholar
  190. Mirabello L, Yeager M, Mai PL, Gastier-Foster JM, Gorlick R, Khanna C, Patino-Garcia A, Sierrasesumaga L, Lecanda F, Andrulis IL, Wunder JS, Gokgoz N, Barkauskas DA, Zhang X, Vogt A, Jones K, Boland JF, Chanock SJ, Savage SA (2015) Germline TP53 variants and susceptibility to osteosarcoma. J Natl Cancer Inst 107(7)Google Scholar
  191. Mody RJ, Bui C, Hutchinson RJ, Yanik GA, Castle VP, Frey KA, Shulkin BL (2010) FDG PET imaging of childhood sarcomas. Pediatr Blood Cancer 54:222–227PubMedPubMedCentralGoogle Scholar
  192. Mukhtar S, Woodhouse C (2010) The management of cyclophosphamide-induced haematuria. BJU Int 105:908–912PubMedGoogle Scholar
  193. Mullen JT, Hornicek FJ, Harmon DC, Raskin KA, Chen YL, Szymonifka J, Yeap BY, Choy E, Delaney TF, Nielsen GP (2014) Prognostic significance of treatment-induced pathologic necrosis in extremity and truncal soft tissue sarcoma after neoadjuvant chemoradiotherapy. Cancer 120:3676–3682PubMedPubMedCentralGoogle Scholar
  194. Mullen JT, Kobayashi W, Wang JJ, Harmon DC, Choy E, Hornicek FJ, Rosenberg AE, Chen YL, Spiro IJ, Delaney TF (2012) Long-term follow-up of patients treated with neoadjuvant chemotherapy and radiotherapy for large, extremity soft tissue sarcomas. Cancer 118:3758–3765PubMedGoogle Scholar
  195. Mundt AJ, Awan A, Sibley GS, Simon M, Rubin SJ, Samuels B, Wong W, Beckett M, Vijayakumar S, Weichselbaum RR (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 32:977–985PubMedGoogle Scholar
  196. Nag S, Martinez-Monge R, Ruymann F, Jamil A, Bauer C (1997) Innovation in the management of soft tissue sarcomas in infants and young children: high-dose-rate brachytherapy. J Clin Oncol 15:3075–3084PubMedGoogle Scholar
  197. Nagasubramanian R, Wei J, Gordon P, Rastatter JC, Cox MC, Pappo A (2016) Infantile Fibrosarcoma With NTRK3-ETV6 Fusion Successfully Treated With the Tropomyosin-Related Kinase Inhibitor LOXO-101. Pediatr Blood Cancer 63:1468–1470PubMedPubMedCentralGoogle Scholar
  198. Navarria P, Ascolese AM, Cozzi L, Tomatis S, D’agostino GR, De Rose F, De Sanctis R, Marrari A, Santoro A, Fogliata A, Cariboni U, Alloisio M, Quagliuolo V, Scorsetti M (2015) Stereotactic body radiation therapy for lung metastases from soft tissue sarcoma. Eur J Cancer 51:668–674PubMedGoogle Scholar
  199. Neglia JP, Friedman DL, Yasui Y, Mertens AC, Hammond S, Stovall M, Donaldson SS, Meadows AT, Robison LL (2001) Second malignant neoplasms in five-year survivors of childhood cancer: childhood cancer survivor study. J Natl Cancer Inst 93:618–629PubMedGoogle Scholar
  200. Ness KK, Hudson MM, Ginsberg JP, Nagarajan R, Kaste SC, Marina N, Whitton J, Robison LL, Gurney JG (2009) Physical performance limitations in the Childhood Cancer Survivor Study cohort. J Clin Oncol 27:2382–2389PubMedPubMedCentralGoogle Scholar
  201. Neuville A, Chibon F, Coindre JM (2014) Grading of soft tissue sarcomas: from histological to molecular assessment. Pathology 46:113–120PubMedGoogle Scholar
  202. Neville HL, Andrassy RJ, Lally KP, Corpron C, Ross MI (2000) Lymphatic mapping with sentinel node biopsy in pediatric patients. J Pediatr Surg 35:961–964PubMedGoogle Scholar
  203. Newton WA Jr, Meadows AT, Shimada H, Bunin GR, Vawter GF (1991) Bone sarcomas as second malignant neoplasms following childhood cancer. Cancer 67:193–201PubMedGoogle Scholar
  204. Nieuwenhuis MH, Lefevre JH, Bulow S, Jarvinen H, Bertario L, Kerneis S, Parc Y, Vasen HF (2011) Family history, surgery, and APC mutation are risk factors for desmoid tumors in familial adenomatous polyposis: an international cohort study. Dis Colon Rectum 54:1229–1234PubMedGoogle Scholar
  205. Nuyttens JJ, Rust PF, Thomas CR Jr, Turrisi AT 3rd (2000) Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: a comparative review of 22 articles. Cancer 88:1517–1523PubMedGoogle Scholar
  206. O’donnell PW, Manivel JC, Cheng EY, Clohisy DR (2014) Chemotherapy influences the pseudocapsule composition in soft tissue sarcomas. Clin Orthop Relat Res 472:849–855PubMedGoogle Scholar
  207. 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 359:2235–2241PubMedGoogle Scholar
  208. Oberlin O, Fawaz O, Rey A, Niaudet P, Ridola V, Orbach D, Bergeron C, Defachelles AS, Gentet JC, Schmitt C, Rubie H, Munzer M, Plantaz D, Deville A, Minard V, Corradini N, Leverger G, De Vathaire F (2009) Long-term evaluation of Ifosfamide-related nephrotoxicity in children. J Clin Oncol 27:5350–5355PubMedGoogle Scholar
  209. Ognjanovic S, Olivier M, Bergemann TL, Hainaut P (2012) Sarcomas in TP53 germline mutation carriers: a review of the IARC TP53 database. Cancer 118:1387–1396PubMedGoogle Scholar
  210. Okcu MF, Munsell M, Treuner J, Mattke A, Pappo A, Cain A, Ferrari A, Casanova M, Ozkan A, Raney B (2003) Synovial sarcoma of childhood and adolescence: a multicenter, multivariate analysis of outcome. J Clin Oncol 21:1602–1611PubMedGoogle Scholar
  211. Orbach D, McDowell H, Rey A, Bouvet N, Kelsey A, Stevens MC (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 57:1130–1136PubMedGoogle Scholar
  212. Overholtzer M, Rao PH, Favis R, Lu XY, Elowitz MB, Barany F, Ladanyi M, Gorlick R, Levine AJ (2003) The presence of p53 mutations in human osteosarcomas correlates with high levels of genomic instability. Proc Natl Acad Sci U S A 100:11547–11552PubMedPubMedCentralGoogle Scholar
  213. Pappo AS, Devidas M, Jenkins J, Rao B, Marcus R, Thomas P, Gebhardt M, Pratt C, Grier HE (2005) Phase II trial of neoadjuvant vincristine, ifosfamide, and doxorubicin with granulocyte colony-stimulating factor support in children and adolescents with advanced-stage nonrhabdomyosarcomatous soft tissue sarcomas: a Pediatric Oncology Group Study. J Clin Oncol 23:4031–4038PubMedGoogle Scholar
  214. Pappo AS, Parham DM, Rao BN, Lobe TE (1999a) Soft tissue sarcomas in children. Semin Surg Oncol 16:121–143PubMedGoogle Scholar
  215. Pappo AS, Rao BN, Jenkins JJ, Merchant T, Poquette CA, Cain A, Pratt CB (1999b) Metastatic nonrhabdomyosarcomatous soft-tissue sarcomas in children and adolescents: the St. Jude Children’s Research Hospital experience. Med Pediatr Oncol 33:76–82PubMedGoogle Scholar
  216. Parham DM, Webber BL, Jenkins JJ 3rd, Cantor AB, Maurer HM (1995) Nonrhabdomyosarcomatous soft tissue sarcomas of childhood: formulation of a simplified system for grading. Mod Pathol 8:705–710PubMedGoogle Scholar
  217. Paulino AC (2004) Late effects of radiotherapy for pediatric extremity sarcomas. Int J Radiat Oncol Biol Phys 60:265–274PubMedGoogle Scholar
  218. Pearlstone DB, Janjan NA, Feig BW, Yasko AW, Hunt KK, Pollock RE, Lawyer A, Horton J, Pisters PW (1999) Re-resection with brachytherapy for locally recurrent soft tissue sarcoma arising in a previously radiated field. Cancer J Sci Am 5:26–33PubMedGoogle Scholar
  219. Pervaiz N, Colterjohn N, Farrokhyar F, Tozer R, Figueredo A, Ghert M (2008) A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer 113:573–581PubMedGoogle Scholar
  220. Pisters PW, Harrison LB, Leung DH, Woodruff JM, Casper ES, Brennan MF (1996a) Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. J Clin Oncol 14:859–868PubMedGoogle Scholar
  221. Pisters PW, Harrison LB, Woodruff JM, Gaynor JJ, Brennan MF (1994) A prospective randomized trial of adjuvant brachytherapy in the management of low-grade soft tissue sarcomas of the extremity and superficial trunk. J Clin Oncol 12:1150–1155PubMedGoogle Scholar
  222. Pisters PW, Leung DH, Woodruff J, Shi W, Brennan MF (1996b) Analysis of prognostic factors in 1,041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol 14:1679–1689PubMedGoogle Scholar
  223. Pisters PW, Pollock RE, Lewis VO, Yasko AW, Cormier JN, Respondek PM, Feig BW, Hunt KK, Lin PP, Zagars G, Wei C, Ballo MT (2007) Long-term results of prospective trial of surgery alone with selective use of radiation for patients with T1 extremity and trunk soft tissue sarcomas. Ann Surg 246:675–681; discussion 681–2PubMedGoogle Scholar
  224. Pratt CB, Maurer HM, Gieser P, Salzberg A, Rao BN, Parham D, Thomas PR, Marcus RB, Cantor A, Pick T, Green D, Neff J, Jenkins JJ (1998) Treatment of unresectable or metastatic pediatric soft tissue sarcomas with surgery, irradiation, and chemotherapy: a Pediatric Oncology Group study. Med Pediatr Oncol 30:201–209PubMedGoogle Scholar
  225. Pratt CB, Pappo AS, Gieser P, Jenkins JJ, Salzbergdagger A, Neff J, Rao B, Green D, Thomas P, Marcus R, Parham D, Maurer H (1999) Role of adjuvant chemotherapy in the treatment of surgically resected pediatric nonrhabdomyosarcomatous soft tissue sarcomas: a Pediatric Oncology Group Study. J Clin Oncol 17:1219PubMedGoogle Scholar
  226. Presant CA, Russell WO, Alexander RW, Fu YS (1986) Soft-tissue and bone sarcoma histopathology peer review: the frequency of disagreement in diagnosis and the need for second pathology opinions. The Southeastern Cancer Study Group experience. J Clin Oncol 4:1658–1661PubMedGoogle Scholar
  227. Radaelli S, Stacchiotti S, Casali PG, Gronchi A (2014) Emerging therapies for adult soft tissue sarcoma. Expert Rev Anticancer Ther 14:689–704PubMedGoogle Scholar
  228. Raney B, Schnaufer L, Ziegler M, Chatten J, Littman P, Jarrett P (1987) Treatment of children with neurogenic sarcoma. Experience at the Children’s Hospital of Philadelphia, 1958–1984. Cancer 59:1–5PubMedGoogle Scholar
  229. Raney RB, Anderson JR, Kollath J, Vassilopoulou-Sellin R, Klein MJ, Heyn R, Glicksman AS, Wharam M, Crist WM, Maurer HM (2000) Late effects of therapy in 94 patients with localized rhabdomyosarcoma of the orbit: report from the Intergroup Rhabdomyosarcoma Study (IRS)-Iii, 1984–1991. Med Pediatr Oncol 34:413–420PubMedGoogle Scholar
  230. Raney RB, Asmar L, Newton WA Jr, Bagwell C, Breneman JC, Crist W, Gehan EA, Webber B, Wharam M, Wiener ES, Anderson JR, Maurer HM (1997) Ewing’s sarcoma of soft tissues in childhood: a report from the Intergroup Rhabdomyosarcoma Study, 1972 to 1991. J Clin Oncol 15:574–582PubMedGoogle Scholar
  231. Raney RB, Asmar L, Vassilopoulou-Sellin R, Klein MJ, Donaldson SS, Green J, Heyn R, Wharam M, Glicksman AS, Gehan EA, Anderson J, Maurer HM (1999) Late complications of therapy in 213 children with localized, nonorbital soft-tissue sarcoma of the head and neck: A descriptive report from the Intergroup Rhabdomyosarcoma Studies (IRS)-II and -III. IRS Group of the Children’s Cancer Group and the Pediatric Oncology Group. Med Pediatr Oncol 33:362–371PubMedGoogle Scholar
  232. Raney RB Jr, Littman P, Jarrett P, Waldman MT, Chatten J (1979) Results of multimodal therapy for children with neurogenic sarcoma. Med Pediatr Oncol 7:229–236PubMedGoogle Scholar
  233. Reitamo JJ, Scheinin TM, Hayry P (1986) The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor. Am J Surg 151:230–237PubMedGoogle Scholar
  234. Reza J, Sammann A, Jin C, Horvai A, Hudnall M, Jablons DM, Jahan T, Kornak J, Mann MJ (2014) Aggressive and minimally invasive surgery for pulmonary metastasis of sarcoma. J Thorac Cardiovasc Surg 147:1193–1200; discussion 1200–1PubMedGoogle Scholar
  235. Rikhof B, De Jong S, Suurmeijer AJ, Meijer C, Van der Graaf WT (2009) The insulin-like growth factor system and sarcomas. J Pathol 217:469–482PubMedGoogle Scholar
  236. Roberts KB, Halperin EC (2011) Soft tissue sarcomas other than rhabdomyosarcoma; desmoid tumor. In: Halperin EC, Constine LS, Tabell JJ, Kun LE (eds) Pediatric radiation oncology. Lippincott Williams & Wilkins, Philadelphia, PAGoogle Scholar
  237. Romeo S, Dei Tos AP (2011) Clinical application of molecular pathology in sarcomas. Curr Opin Oncol 23:379–384PubMedGoogle Scholar
  238. Rosen G, Forscher C, Lowenbraun S, Eilber F, Eckardt J, Holmes C, Fu YS (1994) Synovial sarcoma. Uniform response of metastases to high dose ifosfamide. Cancer 73:2506–2511PubMedGoogle Scholar
  239. Rosenberg SA, Tepper J, Glatstein E, Costa J, Baker A, Brennan M, Demoss EV, Seipp C, Sindelar WF, Sugarbaker P, Wesley R (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 196:305–315PubMedPubMedCentralGoogle Scholar
  240. Rubin BP, Fletcher CD, Inwards C, Montag AG, Peabody T, Qualman SJ, Rosenberg AE, Weiss S, Krausz T (2006) Protocol for the examination of specimens from patients with soft tissue tumors of intermediate malignant potential, malignant soft tissue tumors, and benign/locally aggressive and malignant bone tumors. Arch Pathol Lab Med 130:1616–1629PubMedGoogle Scholar
  241. Rubin BP, Goldblum JR (2007) Pathology of soft tissue sarcoma. J Natl Compr Cancer Netw 5:411–418Google Scholar
  242. Russell WO, Cohen J, Enzinger F, Hajdu SI, Heise H, Martin RG, Meissner W, Miller WT, Schmitz RL, Suit HD (1977) A clinical and pathological staging system for soft tissue sarcomas. Cancer 40:1562–1570PubMedGoogle Scholar
  243. Rutenberg MS, Indelicato DJ, Knapik JA, Lagmay JP, Morris C, Zlotecki RA, Scarborough MT, Gibbs CP, Marcus RB (2011) External-beam radiotherapy for pediatric and young adult desmoid tumors. Pediatr Blood Cancer 57:435–442PubMedGoogle Scholar
  244. Sadoski C, Suit HD, Rosenberg A, Mankin H, Efird J (1993) Preoperative radiation, surgical margins, and local control of extremity sarcomas of soft tissues. J Surg Oncol 52:223–230PubMedGoogle Scholar
  245. Sargar K, Kao SC, Spunt SL, Hawkins DS, Parham DM, Coffin C, Mccarville MB (2015) MRI and CT of low-grade fibromyxoid sarcoma in children: a report from Children’s Oncology Group study ARST0332. AJR Am J Roentgenol 205:414–420PubMedPubMedCentralGoogle Scholar
  246. Scheer M, Dantonello T, Hallmen E, Vokuhl C, Leuschner I, Sparber-Sauer M, Kazanowska B, Niggli F, Ladenstein R, Bielack SS, Klingebiel T, Koscielniak E (2016) Primary metastatic synovial sarcoma: experience of the CWS study group. Pediatr Blood Cancer 63:1198–1206PubMedGoogle Scholar
  247. Schoffski P, Chawla S, Maki RG, Italiano A, Gelderblom H, Choy E, Grignani G, Camargo V, Bauer S, Rha SY, Blay JY, Hohenberger P, D’adamo D, Guo M, Chmielowski B, Le Cesne A, Demetri GD, Patel SR (2016) Eribulin versus dacarbazine in previously treated patients with advanced liposarcoma or leiomyosarcoma: a randomised, open-label, multicentre, phase 3 trial. Lancet 387:1629–1637PubMedGoogle Scholar
  248. Schwartz GK, Tap WD, Qin LX, Livingston MB, Undevia SD, Chmielowski B, Agulnik M, Schuetze SM, Reed DR, Okuno SH, Ludwig JA, Keedy V, Rietschel P, Kraft AS, Adkins D, Van Tine BA, Brockstein B, Yim V, Bitas C, Abdullah A, Antonescu CR, Condy M, Dickson MA, Vasudeva SD, Ho AL, Doyle LA, Chen HX, Maki RG (2013) Cixutumumab and temsirolimus for patients with bone and soft-tissue sarcoma: a multicentre, open-label, phase 2 trial. Lancet Oncol 14:371–382PubMedPubMedCentralGoogle Scholar
  249. SEER. SEER Stat Fact Sheets: Soft Tissue including Heart Cancer [Online]. Accessed 14 June 2016
  250. SEER (1999) Cancer incidence and survival among children and adolescents: United States SEER program 1975–1995. NIH Pub. No. 99–4649 [Online]. Bethesda, MD. 1999: Reis, LAG; Smith, MA; Gurney, JG; Linet, M; Tamra, T; Young, JL; Bunin, GR (eds.) National Cancer Institute, SEER Program. Accessed 14 June 2016
  251. Senthamizhchelvan S, Hobbs RF, Song H, Frey EC, Zhang Z, Armour E, Wahl RL, Loeb DM, Sgouros G (2012) Tumor dosimetry and response for 153Sm-ethylenediamine tetramethylene phosphonic acid therapy of high-risk osteosarcoma. J Nucl Med 53:215–224PubMedPubMedCentralGoogle Scholar
  252. Sherman KL, Kinnier CV, Farina DA, Wayne JD, Laskin WB, Agulnik M, Attar S, Hayes JP, Peabody T, Bilimoria KY (2014) Examination of national lymph node evaluation practices for adult extremity soft tissue sarcoma. J Surg Oncol 110:682–688PubMedGoogle Scholar
  253. Shukla N, Schiffman J, Reed D, Davis IJ, Womer RB, Lessnick SL, Lawlor ER (2013) Biomarkers in Ewing Sarcoma: the promise and challenge of personalized medicine. A report from the Children’s Oncology Group. Front Oncol 3:141PubMedPubMedCentralGoogle Scholar
  254. Siegel RL, Miller KD, Jemal A (2016) Cancer statistics, 2016. CA Cancer J Clin 66:7–30PubMedGoogle Scholar
  255. Silber JH, Littman PS, Meadows AT (1990) Stature loss following skeletal irradiation for childhood cancer. J Clin Oncol 8:304–312PubMedGoogle Scholar
  256. Sindelar WF, Kinsella TJ, Chen PW, Delaney TF, Tepper JE, Rosenberg SA, Glatstein E (1993) Intraoperative radiotherapy in retroperitoneal sarcomas. Final results of a prospective, randomized, clinical trial. Arch Surg 128:402–410PubMedGoogle Scholar
  257. Skapek SX, Ferguson WS, Granowetter L, Devidas M, Perez-Atayde AR, Dehner LP, Hoffer FA, Speights R, Gebhardt MC, Dahl GV, Grier HE (2007) Vinblastine and methotrexate for desmoid fibromatosis in children: results of a Pediatric Oncology Group Phase II Trial. J Clin Oncol 25:501–506PubMedGoogle Scholar
  258. Sklar CA, Mertens AC, Mitby P, Whitton J, Stovall M, Kasper C, Mulder J, Green D, Nicholson HS, Yasui Y, Robison LL (2006) Premature menopause in survivors of childhood cancer: a report from the childhood cancer survivor study. J Natl Cancer Inst 98:890–896PubMedGoogle Scholar
  259. Skubitz KM, D’adamo DR (2007) Sarcoma. Mayo Clin Proc 82:1409–1432PubMedGoogle Scholar
  260. Smith KB, Indelicato DJ, Knapik JA, Lagmay JP, Morris C, Kirwan JM, Zlotecki RA, Scarborough MT, Gibbs CP, Marcus RB (2011a) Adjuvant radiotherapy for pediatric and young adult nonrhabdomyosarcoma soft-tissue sarcoma. Int J Radiat Oncol Biol Phys 81:150–157PubMedGoogle Scholar
  261. Smith KB, Indelicato DJ, Knapik JA, Morris C, Kirwan J, Zlotecki RA, Scarborough MT, Gibbs CP, Marcus RB (2011b) Definitive radiotherapy for unresectable pediatric and young adult nonrhabdomyosarcoma soft tissue sarcoma. Pediatr Blood Cancer 57:247–251PubMedGoogle Scholar
  262. Smith LM, Watterson J, Scott SM (1997) Medical and surgical management of pediatric soft tissue tumors. In: Coffin CM, Dehner LP, O’shea PA (eds) Pediatric soft tissue tumors: a clinical, pathological, and therapeutic approach. Williams and Wilkins, Baltimore, MDGoogle Scholar
  263. Sole CV, Calvo FA, Polo A, Cambeiro M, Alvarez A, Gonzalez C, Gonzalez J, San Julian M, Martinez-Monge R (2014) Anticipated intraoperative electron beam boost, external beam radiation therapy, and limb-sparing surgical resection for patients with pediatric soft-tissue sarcomas of the extremity: a multicentric pooled analysis of long-term outcomes. Int J Radiat Oncol Biol Phys 90:172–180PubMedGoogle Scholar
  264. Sole CV, Calvo FA, Polo A, Cambeiro M, Gonzalez C, Desco M, Martinez-Monge R (2015) Intraoperative electron-beam radiation therapy for pediatric Ewing sarcomas and rhabdomyosarcomas: long-term outcomes. Int J Radiat Oncol Biol Phys 92:1069–1076PubMedGoogle Scholar
  265. Soto-Miranda MA, Sandoval JA, Rao B, Neel M, Krasin M, Spunt S, Jenkins JJ, Davidoff AM, Ver Halen JP (2013) Surgical treatment of pediatric desmoid tumors. A 12-year, single-center experience. Ann Surg Oncol 20:3384–3390PubMedGoogle Scholar
  266. Spunt SL, Hill DA, Motosue AM, Billups CA, Cain AM, Rao BN, Pratt CB, Merchant TE, Pappo AS (2002) Clinical features and outcome of initially unresected nonmetastatic pediatric nonrhabdomyosarcoma soft tissue sarcoma. J Clin Oncol 20:3225–3235PubMedGoogle Scholar
  267. Spunt SL, Million L, Anderson JR et al (2014) Risk-based treatment for nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) in patients under 30 years of age: Children’s Oncology Group study ARST0332 [Abstract]. Last accessed June 27, 2016. J Clin Oncol (5s):32, 10008Google Scholar
  268. Spunt SL, Million L, Coffin C (2015) The nonrhabdomyosarcoma soft tissue sarcoma. In: Pizzo PA, Poplack DG (eds) Principles and practice of pediatric oncology. Wolters Kluwer, Alphen aan den RijnGoogle Scholar
  269. Spunt SL, Pappo AS (2006) Childhood nonrhabdomyosarcoma soft tissue sarcomas are not adult-type tumors. J Clin Oncol 24:1958–1959; author reply 1959–60PubMedGoogle Scholar
  270. Spunt SL, Poquette CA, Hurt YS, Cain AM, Rao BN, Merchant TE, Jenkins JJ, Santana VM, Pratt CB, Pappo AS (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 17:3697–3705PubMedGoogle Scholar
  271. Spunt SL, Skapek SX, Coffin CM (2008) Pediatric nonrhabdomyosarcoma soft tissue sarcomas. Oncologist 13:668–678PubMedGoogle Scholar
  272. Stacchiotti S, Tamborini E, Marrari A, Brich S, Rota SA, Orsenigo M, Crippa F, Morosi C, Gronchi A, Pierotti MA, Casali PG, Pilotti S (2009) Response to sunitinib malate in advanced alveolar soft part sarcoma. Clin Cancer Res 15:1096–1104PubMedGoogle Scholar
  273. Stanelle EJ, Christison-Lagay ER, Wolden SL, Meyers PA, La Quaglia MP (2013) Pulmonary metastasectomy in pediatric/adolescent patients with synovial sarcoma: an institutional review. J Pediatr Surg 48:757–763PubMedGoogle Scholar
  274. Stark AM, Buhl R, Hugo HH, Mehdorn HM (2001) Malignant peripheral nerve sheath tumours—report of 8 cases and review of the literature. Acta Neurochir 143:357–363; discussion 363–4PubMedGoogle Scholar
  275. Stohr W, Paulides M, Bielack S, Jurgens H, Treuner J, Rossi R, Langer T, Beck JD (2007) Ifosfamide-induced nephrotoxicity in 593 sarcoma patients: a report from the Late effects surveillance system. Pediatr Blood Cancer 48:447–452PubMedGoogle Scholar
  276. Suit H, Spiro I (2001) Radiation in the multidisciplinary management of desmoid tumors. Front Radiat Ther Oncol 35:107–119PubMedGoogle Scholar
  277. Tateishi U, Hosono A, Makimoto A, Sakurada A, Terauchi T, Arai Y, Imai Y, Kim EE (2007) Accuracy of 18F fluorodeoxyglucose positron emission tomography/computed tomography in staging of pediatric sarcomas. J Pediatr Hematol Oncol 29:608–612PubMedGoogle Scholar
  278. Taylor BS, Barretina J, Maki RG, Antonescu CR, Singer S, Ladanyi M (2011) Advances in sarcoma genomics and new therapeutic targets. Nat Rev Cancer 11:541–557PubMedPubMedCentralGoogle Scholar
  279. Tepper JE, Suit HD (1985) Radiation therapy alone for sarcoma of soft tissue. Cancer 56:475–479PubMedGoogle Scholar
  280. Thirman MJ, Larson RA (1996) Therapy-related myeloid leukemia. Hematol Oncol Clin North Am 10:293–320PubMedGoogle Scholar
  281. Toma S, Canavese G, Grimaldi A, Ravera G, Ugolini D, Percivale P, Badellino F (2003) Concomitant chemo-radiotherapy in the treatment of locally advanced and/or metastatic soft tissue sarcomas: experience of the National Cancer Institute of Genoa. Oncol Rep 10:641–647PubMedGoogle Scholar
  282. Torres MA, Ballo MT, Butler CE, Feig BW, Cormier JN, Lewis VO, Pollock RE, Pisters PW, Zagars GK (2007) Management of locally recurrent soft-tissue sarcoma after prior surgery and radiation therapy. Int J Radiat Oncol Biol Phys 67:1124–1129PubMedGoogle Scholar
  283. Trojani M, Contesso G, Coindre JM, Rouesse J, Bui NB, De Mascarel A, Goussot JF, David M, Bonichon F, Lagarde C (1984) Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer 33:37–42PubMedGoogle Scholar
  284. Van Dalen EC, Van der Pal HJ, Kok WE, Caron HN, Kremer LC (2006) Clinical heart failure in a cohort of children treated with anthracyclines: a long-term follow-up study. Eur J Cancer 42:3191–3198PubMedGoogle Scholar
  285. Van Der Graaf WT, Blay JY, Chawla SP, Kim DW, Bui-Nguyen B, Casali PG, Schoffski P, Aglietta M, Staddon AP, Beppu Y, Le Cesne A, Gelderblom H, Judson IR, Araki N, Ouali M, Marreaud S, Hodge R, Dewji MR, Coens C, Demetri GD, Fletcher CD, Dei Tos AP, Hohenberger P (2012) Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet 379:1879–1886PubMedGoogle Scholar
  286. Van Der Pal HJ, Van Dalen EC, Van Delden E, Van Dijk IW, Kok WE, Geskus RB, Sieswerda E, Oldenburger F, Koning CC, Van Leeuwen FE, Caron HN, Kremer LC (2012) High risk of symptomatic cardiac events in childhood cancer survivors. J Clin Oncol 30:1429–1437PubMedGoogle Scholar
  287. Viani GA, Novaes PE, Jacinto AA, Antonelli CB, Pellizzon AC, Saito EY, Salvajoli JV (2008) High-dose-rate brachytherapy for soft tissue sarcoma in children: a single institution experience. Radiat Oncol 3:9PubMedPubMedCentralGoogle Scholar
  288. Wagner LM, Neel MD, Pappo AS, Merchant TE, Poquette CA, Rao BN, Rodriguez-Galindo C (2001) Fractures in pediatric Ewing sarcoma. J Pediatr Hematol Oncol 23:568–571PubMedGoogle Scholar
  289. Wang D, Zhang Q, Eisenberg BL, Kane JM, Li XA, Lucas D, Petersen IA, Delaney TF, Freeman CR, Finkelstein SE, Hitchcock YJ, Bedi M, Singh AK, Dundas G, Kirsch DG (2015) Significant reduction of late toxicities in patients with extremity sarcoma treated with image-guided radiation therapy to a reduced target volume: results of Radiation Therapy Oncology Group RTOG-0630 trial. J Clin Oncol 33:2231–2238PubMedPubMedCentralGoogle Scholar
  290. Waxweiler TV, Rusthoven CG, Proper MS, Cost CR, Cost NG, Donaldson N, Garrington T, Greffe BS, Heare T, Macy ME, Liu AK (2015) Non-rhabdomyosarcoma soft tissue sarcomas in children: a surveillance, epidemiology, and end results analysis validating COG risk stratifications. Int J Radiat Oncol Biol Phys 92:339–348PubMedGoogle Scholar
  291. Weiser MR, Downey RJ, Leung DH, Brennan MF (2000) Repeat resection of pulmonary metastases in patients with soft-tissue sarcoma. J Am Coll Surg 191:184–190; discussion 190–1PubMedGoogle Scholar
  292. Weiss A (2014) Radiation therapy with or without combination chemotherapy or pazopanib hydrochloride before surgery in treating patients with newly diagnosed non-rhabdomyosarcoma soft tissue sarcomas that can be removed by surgery. Identifier: NCT02180867
  293. Weiss SW, Goldblum JR (2008) Miscellaneous tumors of intermediate malignancy. In: Weiss SW, Goldblum JR (eds) Enzinger and Weiss’s soft tissue tumors. Mosby Building Arts, St. Louis, MOGoogle Scholar
  294. Weyl Ben Arush M, Meller I, Moses M, Klausner J, Isakov J, Kuten A, El Hassid R (1998) Multifocal desmoid tumor in childhood: report of two cases and review of the literature. Pediatr Hematol Oncol 15:55–61PubMedGoogle Scholar
  295. Wolden SL (2005) Radiation therapy for non-rhabdomyosarcoma soft tissue sarcomas in adolescents and young adults. J Pediatr Hematol Oncol 27:212–214PubMedGoogle Scholar
  296. Woll PJ, Reichardt P, Le Cesne A, Bonvalot S, Azzarelli A, Hoekstra HJ, Leahy M, Van Coevorden F, Verweij J, Hogendoorn PC, Ouali M, Marreaud S, Bramwell VH, Hohenberger P (2012) Adjuvant chemotherapy with doxorubicin, ifosfamide, and lenograstim for resected soft-tissue sarcoma (EORTC 62931): a multicentre randomised controlled trial. Lancet Oncol 13:1045–1054PubMedGoogle Scholar
  297. Womer RB, West DC, Krailo MD, Dickman PS, Pawel BR, Grier HE, Marcus K, Sailer S, Healey JH, Dormans JP, Weiss AR (2012) Randomized controlled trial of interval-compressed chemotherapy for the treatment of localized Ewing sarcoma: a report from the Children’s Oncology Group. J Clin Oncol 30:4148–4154PubMedPubMedCentralGoogle Scholar
  298. Worden FP, Taylor JM, Biermann JS, Sondak VK, Leu KM, Chugh R, Mcginn CJ, Zalupski MM, Baker LH (2005) Randomized phase II evaluation of 6 g/m2 of ifosfamide plus doxorubicin and granulocyte colony-stimulating factor (G-CSF) compared with 12 g/m2 of ifosfamide plus doxorubicin and G-CSF in the treatment of poor-prognosis soft tissue sarcoma. J Clin Oncol 23:105–112PubMedGoogle Scholar
  299. 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 16:197–203PubMedGoogle Scholar
  300. Yock T (2007) Proton radiation for the treatment of pediatric bone and non-rhabdomyosarcoma soft tissue sarcomas. Identifier: NCT00592293.
  301. Zagars GK, Ballo MT (2003) Significance of dose in postoperative radiotherapy for soft tissue sarcoma. Int J Radiat Oncol Biol Phys 56:473–481PubMedGoogle Scholar
  302. Zagars GK, Ballo MT, Pisters PW, Pollock RE, Patel SR, Benjamin RS (2003) Prognostic factors for disease-specific survival after first relapse of soft-tissue sarcoma: analysis of 402 patients with disease relapse after initial conservative surgery and radiotherapy. Int J Radiat Oncol Biol Phys 57:739–747PubMedGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2018

Authors and Affiliations

  1. 1.Department of Radiation OncologySt. Jude Children’s Research HospitalMemphisUSA

Personalised recommendations