Evolving Paradigms in Soft Tissue Sarcoma: Conclusion and References

Evolving ParadigmsSoft Tissue Sarcoma
Volume 2
Issue 1

This section covers the Current Treatment Strategies section of the current Evolving Paradigms in Soft Tissue Sarcoma issue.


Currently, STS is difficult to treat due to its heterogeneity and the lack of available treatments. With advances in the knowledge of genetics, understanding of STS continues to increase. This increase of knowl- edge has promoted, and will continue to promote, the development of targeted therapies, which may aid in personalizing treatment and will help shape future standards of care.


  1. Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, eds.WHO Classification of Tumours of Soft Tissue and Bone. Lyon, France: IARC; 2002.
  2. Forman D, Bray F, Brewster DH, et al, eds. Cancer incidence in five continents. Vol X. Lyon, France: IARC; 2014.
  3. American Cancer Society. Cancer Treatment & Survivorship Facts & Figures 2016-2017. Atlanta: American Cancer Society; 2016. Accessed December 21, 2016.
  4. National Cancer Institute. SEER Stat Fact Sheets. Soft tissue including heart cancer. http:// seer.cancer.gov/statfacts/html/soft.html. Accessed July 2015.
  5. Burningham Z, Hashibe M, Spector L, Schiffman JD. The epidemiology of sarcoma.Clin Sarcoma Res. 2012;2(1):14.
  6. Penel N, Grosjean J, Robin YM, Vanseymortier L, Clisant S, Adenis A. Frequency of certain established risk factors in soft tissue sarcomas in adults: a prospective descriptive study of 658 cases.Sarcoma. 2008:1-6.
  7. Kleinerman RA, Tucker MA, Abramson DH, Seddon JM, Tarone RE, Fraumeni JF Jr. Risk of soft tissue sarcomas by individual subtype in survivors of hereditary retinoblastoma.J Natl Cancer Inst. 2007;99:24-31.
  8. National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: soft tissue sarcoma version 1.2017. http://www.nccn.org. Accessed November 2, 2015.
  9. American Cancer Society. Soft tissue sarcoma. http://www.cancer.org/cancer/sarcoma- adultsofttissuecancer/. Accessed December 22, 2016.
  10. Chen C, Borker R, Ewing J, et al. Epidemiology, treatment patterns, and outcomes of metastatic soft tissue sarcoma in a community-based oncology network.Sarcoma. 2014;2014:145764.
  11. ESMO/European Sarcoma Network Working Group. Soft tissue and visceral sarcomas: ESMO clinical practice guidelines for diagnosis, treatment, and follow-up.Ann Oncol. 2014;25(suppl 3):iii102-iii112.
  12. Wardelmann E, Schildhaus HU, Merkelbach-Bruse S, et al. Soft tissue sarcoma: from molec- ular diagnosis to selection of treatment. Pathological diagnosis of soft tissue sarcoma amid molecular biology and targeted therapies.Ann Oncol. 2010;21(suppl 7):vii265-vii269.
  13. Guillou L, Coindre JM, Bonichon F, et al. 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. 1997;15(1):350-362.
  14. Sorensen PH, Lynch JC, Qualman SJ, et al. PAX-FKHR and PAX7-FKHR gene fusions are prognostic in alveolar rhabdomyosarcoma: a report from the children’s oncology group.J Clin Oncol. 2002;20(11):2672-2679.
  15. Ladanyi M, Antonescu CR, Leung DH, et al. Impact of SYT-SSX fusion type on the clinical behavior of synovial sarcoma: a multi-institutional retrospective study of 243 patients.Cancer Res. 2002; 62(1):135-140.
  16. Sun Y, Sun B, Wany J, et al. Prognostic implication of SYT-SSX fusion type and clinicopatho- logical parameters for tumor-related death, recurrence, and metastasis in synovial sarcoma.Cancer Sci. 2009;100(6):1018-1025.
  17. Guillou L, Benhattar J, Bonichon F, et al. Histologic grade, but not SYT-SSX fusion type, is an important prognostic factor in patients with synovial sarcoma: a multicenter, retrospective analysis.J Clin Oncol. 2004;22:4040-4050.
  18. American Joint Committee on Cancer. What is the TNM staging system? https://cancerstag- ing.org/references-tools/Pages/What-is-Cancer-Staging.aspx. Accessed October 19, 2015.
  19. Daigeler A, Zmarsly I, Hirsch T, et al. Long-term outcome after local recurrence of soft tissue sarcoma: a retrospective analysis of factors predictive of survival in 135 patients with lo- cally recurrent soft tissue sarcoma.Br J Cancer. 2014;110(6):1456-1464.
  20. Corey RM, Swett K, Ward WG. Epidemiology and survivorship of soft tissue sarcomas in adults: a national cancer database report.Cancer Med. 2014;3(5):1404-1415.
  21. Zagars GK, Ballo MT, Pisters PWT, Pollock RE, Patel SR, Benjamin RS. Surgical margins and reresection in the management of patients with soft tissue sarcoma using conservative surgery and radiation therapy.Cancer. 2003;97(10):2544-2553.
  22. Griffin AM, Euler CI, Sharpe MB, et al. Radiation planning comparison for superficial tissue avoidance in radiotherapy for soft tissue sarcoma of the lower extremity.Int J Radiat Oncol Biol Phys. 2007;67(3):847-856.
  23. Folkert MR, Singer S, Brennan MF, et al. Comparison of local recurrence with conventional and intensity-modulated radiation therapy for primary soft-tissue sarcomas of the extremity.J Clin Oncol. 2014;32(29):3236-3241.
  24. Roeder F, Lehner B, Schmitt T, et al. Excellent local control with IOERT and postoperative EBRT in high grade extremity sarcoma: results from a subgroup analysis of a prospective trial.BMC Cancer. 2014;14:350.
  25. Alektiar KM, Brennan MF, Singer S. Local control comparison of adjuvant brachytherapy to intensity-modulated radiotherapy in primary high-grade sarcoma of the extremity.Cancer. 2011;117(4):3229-3234.
  26. Yoon SS, Chen YL, Kirsch DG, et al. Proton-beam, intensity-modulated, and/or intraoperative electron radiation therapy combined with aggressive anterior surgical resection for retro- peritoneal sarcomas.Ann Surg Oncol. 2010;17(6):1515-1529.
  27. Baldini EH, Lapidus MR, Wang Q, et al. Predictors for major wound complications following preoperative radiotherapy and surgery for soft-tissue sarcoma of the extremities and trunk: importance of tumor proximity to skin surface.Ann Surg Oncol. 2013;20(5):1494-1499.
  28. Davis AM, O’Sullivan B, Turcotte R, et al. ;Canadian Sarcoma Group; NCI Canada Clinical Trial Group Randomized Trial. Late radiation morbidity following randomization to preop- erative versus postoperative radiotherapy in extremity soft tissue sarcoma.Radiother Oncol. 2005;75(1):48-53.
  29. Al Yami A, Griffin AM, Ferguson PC, et al. Positive surgical margins in soft tissue sarcoma treated with preoperative radiation: is a postoperative boost necessary?Int J Radiat Oncol Biol Phys. 2010;77(4):1191-1197.
  30. Pan E, Goldberg SI, Chen YL, et al. Role of post-operative radiation boost for soft tissue sarcomas with positive margins following pre-operative radiation and surgery.J Surg Oncol. 2014;110(7):817-822.
  31. Holt GE, Griffin AM, Pintilie M, et al. Fractures following radiotherapy and limb-salvage surgery for lower extremity soft-tissue sarcomas. A comparison high-dose and low-dose radiotherapy.J Bone Joint Surg Am. 2005;87(2):315-319.
  32. Dangoor A, Seddon B, Gerrand C, Grimer R, Whelan J, Judson I. UK guidelines for the management of soft tissue sarcomas. Clin Sarcoma Res. 2016;6:20.
  33. Pervaiz N, Colterjohn N, Farrokhyar F, et al. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma.Cancer. 2008;113(3):573-581.
  34. Woll PJ, Reichardt P, Le Cesne A, et al. ;EORTC Soft Tissue and Bone Sarcoma Group and the NCIC Clinical Trials Group Sarcoma Disease Site Committee. Adjuvant chemotherapy with doxorubicin, ifosfamide, and lenograstim for resected soft-tissue sarcoma (EORTC 62931): a multicenter randomized controlled trial.Lancet Oncol. 2012;13(10):1044-1054.
  35. D’Adamo D. Is adjuvant chemotherapy useful for soft-tissue sarcomas?Lancet. 2012;13(10):968-970.
  36. Frustaci S, Gherlizoni F, De Paoli A, Tozer R, Figueredo A, Chert M. Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian random- ized cooperative trial.J Clin Oncol. 2001;19(5):1238-1247.
  37. Italiano A, Delva F, Mathoulin-Pelissier, et al. Effect of adjuvant chemotherapy on survival in FNCLCC grade 3 soft tissue sarcomas: a multivariate analysis of the French Sarcoma Group Database.Ann Oncol. 2010;21(12):2436-2441.
  38. Mullen JT, Kobayashi W, Wang JJ, et al. Long-term follow-up of patients treated with neoad- juvant chemotherapy and radiotherapy for large, extremity soft tissue sarcomas.Cancer. 2012;118(15):3758-3765.
  39. Bramwell VH, Anderson D, Charette ML; Sarcoma Disease Site Group. Doxorubicin-based chemotherapy for the palliative treatment of adult patients with locally advanced or meta- static soft tissue sarcoma.Cochrane Database Syst Rev. 2003;(3):CD003293.
  40. Judson I, Verweij J, Gelderblom H, et al; European Orgnisation and Treatment of Cancer Soft Tissue and Bone Sarcoma Group.. Doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced or metastatic soft-tissue sarcoma: a random- ized controlled phase 3 trial.Lancet Oncol. 2014;15(4):414-423.
  41. Maki RG, Wathen JK, Patel SR, et al. Randomized phase II study of gemcitabine and docetaxel compared with gemcitabine alone in patients with metastatic soft tissue sarco- mas: results of sarcoma alliance for research through collaboration study 002.J Clin Oncol. 2007;25(19):2755-2763.
  42. Pautier P, Floquet A, Penel N, et al. Randomized multicenter and stratified phase II study of gemcitabine alone versus gemcitabine and docetaxel in patients with metastatic or relapsed leiomyosarcomas: a Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) French Sarcoma Group Study (TAXOGEM study).Oncologist. 2012;17(9):1213-1220.
  43. Garcia del Muro X, Lopez-Pousa A, Maurel J, et al; Spanish Group for Research on Sarcomas. 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. 2011;29(18):2528-2533.
  44. Sharma S, Takyar S, Manson SC, Powell S, Penel N. Efficacy and safety of pharmacological interventions in second- or late-line treatment of patients with advance soft tissue sarcoma: a systematic review.BMC. 2013;13:385.
  45. Samuels BL, Chawla S, Patel S, et al. 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. 2013;24(6):1703-1709.
  46. [press release]. Silver Spring, MD: FDA; October 23, 2015. FDA approves new therapy for certain types of advanced soft tissue sarcoma. http://www.fda.gov/NewsEvents/Newsroom/ PressAnnouncements/ucm468832.htm. Accessed November 2, 2015.
  47. Demetri GD, von Mehren M, Jones RL, et al. Efficacy and safety of trabectedin or dacarba- zine for metastatic liposarcoma or leiomyosarcoma after failure of conventional che- motherapy: results of a phase III randomized multicenter clinical trial.J Clin Oncol. [published online September 14, 2015]. pii: JCO.2015.62.4734.
  48. Davis EJ, Chugh R, Patel S, et al. A retrospective analysis of patients with soft tissue sarcoma treated long-term with trabectedin. J Clin Oncol. 2015;33(suppl: abstract 10551).
  49. Blay JY, Leahy MG, Nguyen BB, et al. Randomised phase III trial of trabectedin versus doxorubicin-based chemotherapy as first-line therapy in translocation-related sarco- mas.Eur J Cancer. 2014;50(6):1137-1147.
  50. Yondelis (trabectedin) [prescribing information]. Horsham, PA: Janssen Products, LP; 2015.
  51. Schöffski P, Ray-Coquard IL, Cioffi A, et al ;European Organisation for Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group.. Activity of eribu- lin mesylate in patients with soft-tissue sarcoma: a phase 2 study in four independent histological subtypes.Lancet Oncol. 2011;12(11):1054-1052.
  52. FDA approves first drug to show survival benefit in liposarcoma [press release]. Silver Spring, MD: US Food and Drug Administration; January 28, 2016. http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm483714.htm. Accessed December 22, 2016.
  53. Schöffski P, Maki RG, Italiano A, et al. Randomized, open-label, multicenter, phase III study of eribulin versus dacarbazine in patients (pts) with leiomyosarcomas (LMS) and adipocytic sarcoma (ADI). J Clin Oncol. 2015:33(suppl: abstract LBA 10502).
  54. Schöffski P, Chawla S, Maki RG, et al. Eribulin versus dacarbazine in previously treated patients with advanced liposarcoma or leiomyosarcoma; a randomised, open-labell, multicenter, phase 3 trial. Lancet. 2016;387(10028):1629-1637.
  55. FDA grants accelerated approval to new treatment for advanced soft tissue sarcoma [press release]. Silver Springs, MD; October 19, 2016. http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm525878.htm. Accessed December 22, 2016.
  56. CHMP recommends approval of lilly's olaratumab, in combination with doxorubicin, for advanced soft tissue sarcoma [press release]. Indianapolis, IN; September 16, 2016. https://investor.lilly.com/releasedetail.cfm?ReleaseID=989359. Accessed March 7, 2017.
  57. Tap WD, Jones RL, Van Tine BA, et al. Olaratumab and doxorubicin versus doxorubicin alone for treatment of soft-tissue sarcoma: an open-label phase 1b and randomised phase 2 trial. Lancet. 2016;388(10043):488-497.
  58. Lartruvo (olaratumab) [prescribing information]. Indianapolis, IN: Eli Lilly and Company; 2016.
  59. Brennan M, Antonescu C, Alektiar K, Maki R.Management of Soft Tissue Sarcoma. 2nd ed. Switzerland: Springer International Publishing; 2016.
  60. Olaratumab (LARTRUVO): Approved Drugs. FDA.gov. https://www.fda.gov/Drugs/InformationOnDrugs/ApprovedDrugs/ucm526087.htm. Accessed March 7, 2017.
  61. NCT02451943. A study of doxorubicin plus olaratumab (LY3012207) in participants with advanced or metastatic soft tissue sarcoma (ANNOUNCE). Clinical Trials website. https://clinicaltrials.gov/ct2/show/NCT02451943. Accessed March 7, 2017.
  62. Wilky BA, Meyer CF, Trent JC. Pazopanib in sarcomas: expanding the PALETTE.CurrOpin Oncol. 2013;25(4):373-378.
  63. van der Graaf WT, Blay JY, Chawla SP, et al ;EORTC Soft Tissue and Bone Sarcoma Group; PALETTE study group. Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomized, double-blind, placebo-controlled phase 3 trial.Lancet. 2012;379(9829):1879-1886.
  64. Coens C, van der Graaf WT, Blay JY, et al. Health-related quality-of-life results from PALETTE: a randomized, double-blind, phase 3 trail of pazopanib versus placebo
in patients with soft tissue sarcoma whose disease has progressed during or after prior chemotherapy—a European Organization for research and treatment of cancers soft tissue and bone sarcoma group global network study (EORTC 62072).Cancer. 2015;121(17):2933-2941.
  65. Kasper B, Sleijfer S, Litière S, et al. Long-term responders and survivors on pazopanib for advanced soft tissue sarcomas: subanalysis of two European Organisation for Research Treatment of Cancer (EORTC) clinical trials 62034 and 62072.Ann Oncol. 2014;25(3):719-724.
  66. Desar IME, Constantinidou A, Kaal SEJ, et al. Advanced soft-tissue sarcoma and treatment options: critical appraisal of trabectedin. Cancer Manag Res. 2016;8:95-104. doi: 10.2147/CMAR.S86746.
  67. Mir O, Domont J, Cioffi A, et al. Feasibility of metronomic oral cyclophosphamide plus prednisolone in elderly patients with inoperable or metastatic soft tissue sarcoma. Eur J Cancer. 2011;47(4):515—519.
  68. Lebrecht D, Geist A, Ketelsen UP, et al. The 6-maleimidocaproyl hydrazine derivative of doxorubicin (DOXO-EMCH) is superior to free doxorubicin with respect to cardiotoxicity and mitochondrial damage.Int J Cancer. 2006;120(4).
  69. Chawla SP, Chua VS, Hendifar AF, et al. A phase 1B/2 study of aldoxorubicin in patients with soft tissue sarcoma.Cancer. 2015;121(4):570-579.
  70. Chawla SP, Papai Z, Mukhametshina G, et al. First-line aldoxorubicin vs doxorubicin in metastatic or locally advanced unresectable soft-tissue sarcoma.JAMA Oncol.2015;1(9):1272-1280.
  71. Saggar JK, Tannock IF. Activity of the hypoxia-activated pro-drug TH-302 in hypoxic and perivascular regions of solid tumors and its potential to enhance therapeutic effects of chemotherapy.Int J Cancer. 2013;134(11):2726-2734.
  72. Chawla SP, Cranmer LD, Van Tine BA, et al. Phase II study of the safety and antitumor activity of hypoxia-activated prodrug TH-302 in combination with doxorubicin in patients with advance soft tissue sarcoma.J Clin Oncol. 2014;32(29):3299-3306.
  73. Tap W, Papai Z, van Tine B, et al. Randomized phase 3, multicenter, open-label study comparing evofosfamide (Evo) in combination with doxorubicin (D) vs. D alone in patients (pts) with advanced soft tissue sarcoma (STS): Study TH-CR-406/SARC021. Ann Oncol. 2016;27(suppl 6):1395O.
  74. Tawbi H A, Burgess MA, Crowley J, et al. Safety and efficacy of PD-1 blockade using pembrolizumab in patients with advanced soft tissue (STS) and bone sarcomas (BS): Results of SARC028—A multicenter phase II study. J Clin Oncol. 2016;34 (suppl; abstr 11006).
  75. Paoluzzi L, Ghesani MV, Cacavio A, et al. Anti-PD1 therapy with nivolumab in sarcoma. J Clin Oncol 34, 2016 (suppl; abstr 11047).
  76. NCT02500797. Nivolumab with or without ipilimumab in treating patients with metastatic sarcoma that cannot be removed by surgery. Clinical Trials website. https://clinicaltrials.gov/ct2/show/NCT02500797. Accessed December 28, 2016.
  77. Kim JR, Moon YJ, Kwon KS, et al. Tumor infiltrating PD1-positive lymphocytes and the expression of PD-L1 predict poor prognosis of soft tissue sarcomas. PLoS One. 2013;8(12):e82870
  78. Somaiah N, von Mehren M. New drugs and combinations for the treatment of soft-tissue sarcoma: a review.Cancer Manag Res. 2012;4:397-411.
  79. von Mehren M, Rankin C, Goldblum JR, et al. Phase II Southwest Oncology Group-directed intergroup trial (S0505) of sorafenib in advanced soft tissue sarcomas.Cancer. 2012;118(3):770-776.
  80. Chugh R, Wathen JK, Maki RG, et al. Phase II multicenter trial of imatinib in 10 histologic subtypes of sarcoma using a Bayesian hierarchical statistical model.J Clin Oncol. 2009;27(19):3148-3153.
  81. George S, Merriam P, Maki RG, et al. Multicenter phase II trial sunitinib in the treatment of nongastrointestinal stromal tumor sarcomas.J Clin Oncol. 2009;27(19):3154-3160.
  82. Sugiura H, Fujiwara Y, Ando M, et al. Multicenter phase II trial assessing effectiveness of imatinib mesylate on relapsed or refractory KIT-positive or PDGFR-positive sarcoma.J Orthop Sci. 2010;15(5):654-660.
  83. Maki RG, D’Adamo DR, Keohan ML, et al. Phase II study of sorafenib in patients with metastatic or recurrent sarcomas.J Clin Oncol. 2009;27(19):3133-3140.
  84. Chi Y, Sun Y, Cai J, et al. Phase II study of anlotinib for treatment of advanced soft tissues sarcoma. J Clin Oncol 34, 2016 (suppl; abstr 11005).
  85. Mir O, Brodowicz T, Wallet J, et al. Activity of regorafenib (RE) in leiomyosarcomas (LMS) and other types of soft-tissue sarcomas (OTS): results of a double-blind, ran- domized placebo (PL) controlled phase II trial.J Clin Oncol. 2015;33(suppl: abstract 10504).
  86. Mir O, Brodowicz T, Italiano A, et al. Safety and efficacy of regorafenib in patients with advanced soft tissue sarcoma (REGOSARC): a randomised, double-blind, placebo-controlled, phase 2 trial. Lancet Oncol. 2016;17(12):1732-1742.
  87. Chawla SP, Staddon AP, Baker LH, et al. Phase II study of mammalian target rapamycin inhibitor ridaforolimus in patients with advanced bone and soft tissue sarcomas.J Clin Oncol. 2011;30(1):78-84.
  88. Blay JY, Chawla SP, Ray-Coquard I, et al. Phase III, placebo-controlled trial (SUCCEED) evaluating ridaforolimus as maintenance therapy in advanced sarcoma patients follow- ing clinical benefit from prior standard cytotoxic chemotherapy: long-term (24 months) overall survival.J Clin Oncol. 2012;30 (suppl: abstract 10010).
  89. Wagner AJ, Malinowska-Kolodziej I, Morgan JA, et al. Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors.J Clin Oncol. 2010;28(5):835-840.
  90. Okuno S, Bailey H, Mahoney MR, et al. A phase 2 study of temsirolimus (CCI-779) in patients with soft tissues sarcomas: a study of the Mayo phase 2 consortium (P2C).Cancer. 2011;117(15):3468-3475.
  91. D’Adamo DR, Anderson SE, Albritton K, et al. Phase II study of doxorubicin and bevacizumab for patients with metastatic soft-tissue sarcomas.J Clin Oncol. 2005;23(28):7135-7142.
  92. Schöffski P, Adkins D, Blay JY, et al. An open-label, phase 2 study evaluating the efficacy and safety of the anti-IGF-1R antibody cixutumumab in patients with previously treated advanced or metastatic soft-tissue sarcoma or Ewing family of tumours.Eur J Cancer. 2013;49(15):3219-3228.
  93. Chawla SP, Schoffski P, Blay JY, et al. A study of the safety and efficacy of the combination of gemcitabine and docetaxel with ontuxizumab (MORAb-004) in metastatic soft tissue sarcoma.J Clin Oncol. 2015;33(suppl: abstract TPS 10577).
  94. Hensley ML, Maki R, Venkatraman E, et al. Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma:results of a phase II trial. J Clin Oncol. 2002;20(12):2824-2831.
  95. Pautier P, Floquet A, Penel N, et al. Randomized multicenter and stratified phase II study of gemcitabine alone versus gemcitabine and docetaxel in patients with metastatic or relapsed leiomyosarcomas: a Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) French Sarcoma Group Study (TAXOGEM study).Oncologist. 2012;17(9):1213-1220.
  96. Baker LH, Crowley JJ, Maki RG. Randomization and statistical power: paramount in trial reproducibility (even for rare cancers).Oncologist. 2012;17(9):1129-1132.
  97. Ikeda S, Kudoh K, Sasaki N, et al. Synergistic effects of cabozantinib to temozolomide and bevacizumab in patients with heavily pretreated relapsed uterine leiomyosarcoma.J Clin Oncol. 2015; 22 (suppl: abstract 5590).
  98. Hensley ML, Blessing JA, Mannel R, Rose PG. Fixed-dose rate gemcitabine plus docetaxel as first-line therapy for metastatic uterine leiomyosarcoma: a Gynecologic Oncology Group phase II trial. Gynecol Oncol. 2008;109(3):329—334.
  99. Hensley ML, Miller A, O’Malley DM, et al. Randomized phase III trial of gemcitabine plus docetaxel plus bevacizumab or placebo as first-line treatment for metastatic uterine leiomyosarcoma: an NRG Oncology/Gynecologic Oncology Group study. J Clin Oncol. 2015;33(10):1180—1185.
  100. Hensley ML, Patel SR, Mehren MV, et al. Efficacy and safety of trabectedin or dacarbazine for the treatment of patients with uterine leiomyosarcoma after prior chemotherapy: A subgroup analysis of the randomized phase 3 SAR-3007 study. Presented at: 2016 Society of Gynecologic Oncology Annual Meeting; March 19-22, 2016; San Diego, CA.
  101. Forni C, Minuzzo M, Virdis E, et al. Trabectedin (ET-743) promotes differentiation in myxoid liposarcoma tumors.Mol Cancer Ther. 2009;8(2):449-457.
  102. Grosso F, Jones RL, Demetri GD, et al. Efficacy of trabectedin (ecteinascidin-743) in advanced pretreated myxoid liposarcomas: a retrospective study.Lancet Oncol. 2007;8(7):595-602.
  103. Grosso F, Sanfilippo R, Virdis E, et al. Trabectedin in myxoid liposarcomas (MLS): a long- term analysis of single-institution series.Ann Oncol. 2009;20(8):1439-1444.
  104. Dickson MA, Tap WD, Keohan ML, et al. Phase II trial of the CDK4 inhibitor PD0332991 in patients with advanced CDK4-amplified well-differentiated or dedifferentiated liposarcoma. J Clin Oncol. 2013;31(16):2024-2028.
  105. Orbach D, Brennan B, Casanova M, et al. Paediatric and adolescent alveolar soft part sarcoma: a joint series from European Cooperative Groups.Pediatr Blood Cancer. 2013;60(11):1826-1832.
  106. Portera CA, Ho V, Patel SR, et al. Alveolar soft part sarcoma: clinical course and patterns of metastasis in 70 patients treated at a single institution.Cancer. 2001;91(3):585-591.
  107. Ogose A, Yazawa Y, Ueda T, et al. ;Japanese Musculoskeletal Oncology Group. Alveolar soft part sarcoma in Japan: multi-institutional study of 57 patients from Japanese Musculoskeletal Oncology Group.Oncology. 2003;65(1):7-13.
  108. Reichardt P, Lindner T, Pink D, Thuss-Patience PC, Kretzschmar A, Dörken B. Chemotherapy in alveolar soft part sarcomas. What do we know? Eur J Cancer. 2003;39(11):1511-1516.
  109. Stockwin LH, Vistica DT, Kenney S, et al. Gene expression profiling of alveolar soft-part sarcoma (ASPS).BMC Cancer. 2009;9:22.
  110. Stacchiotti S, Negri T, Zaffaroni N, et al. Sunitinib in advanced alveolar soft part sarcoma: evidence of a direct antitumor effect.Ann Oncol. 2011;22(7):1682-1690.
  111. Kummar S, Allen D, Monks A, et al. Cediranib for metastatic alveolar soft part sarcoma.JClin Oncol. 2013;31(18):2296-2302.
  112. Hong DS, Garrido-Laguna I, Ekmekcioglu S, et al. Dual inhibition of the vascular endothelial growth factor pathway.Cancer. 2014;120(14):2164-2173.
  113. Fayette J, Martin E, Piperno-Neumann S, et al. Angiosarcomas, a heterogeneous group of sarcomas with specific behavior depending on primary site: a retrospective study of 161 cases.Ann Oncol. 2007;18(12):2030-2036.
  114. Fury MG, Antonescu CR, Van Zee KJ, et al. A 14-year retrospective review of angiosarcoma: clinical characteristics, prognostic factors, and treatment outcomes with surgery and chemotherapy.Cancer J. 2005;11(3):241-247.
  115. Stacchiotti S, Negri T, Libertini M, et al. Sunitinib malate in solitary fibrous tumor (SFT).Ann Oncol. 2012;23(12):3171-3179.
  116. Penel N, Bui BN, Bay JO, et al. Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX study.J Clin Oncol.2008;26(32):5269-5274.
  117. Italiano A, Cioffi A, Penel N, et al. Comparison of doxorubicin and weekly paclitaxel efficacy in metastatic angiosarcomas.Cancer. 2012;118(13):3330-3336.
  118. Schlemmer M, Reichardt P, Verweij J, et al. Paclitaxel in patients with advanced angiosarcomas of soft tissue: a retrospective study of the EORTC soft tissue and bone sarcoma group.Eur J Cancer.2008;44(16):2433—2436.
  119. Ray-Coquard I, Italiano A, Bompas E, et al; French Sarcoma Group (GSF/GETO). Sorafenib for patients with advanced angiosarcoma: a phase II trial from the French Sarcoma Group (GSF/GETO).Oncologist. 2012;17(2):260-266.
  120. Agulnik M, Yarber JL, Okuno SH, et al. An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas.Ann Oncol. 2013;24(1):257-263.
  121. National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: dermatofibrosarcoma protuberans version 1.2015. Published December 16, 2014. http://www.nccn.org/professionals/physician_gls/recently_updated.asp. Accessed October 6, 2015.
  122. Rutkowski P, Van Glabbeke M, Rankin CJ, et al ;European Organisation for Research and Treatment of Cancer Soft Tissue/Bone Sarcoma Group. Southwest Oncology Group.. Imatinib mesylate in advanced dermatofibrosarcoma protuberans: pooled analysis of two phase II clinical trials.J Clin Oncol. 2010;28(2):1772-1779.
  123. Kérob D, Porcher R, Verola O, et al. Imatinib mesylate as a preoperative therapy in der- matofibrosarcoma: results of a multicenter phase II study on 25 patients.Clin Cancer Res. 2010;16(12):3288-3295.
  124. Ugurel S, Mentzel T, Utikal J, et al. Neoadjuvant imatinib in advanced primary or lo- cally recurrent dermatofibrosarcoma protuberans: a multicenter phase II DeCOG trial with long-term follow-up.Clin Cancer Res. 2013;20(2):499-510.
  125. Butrynski JE, D’Adamo DR, Hornick JL, et al. Crizotinib in ALK-rearranged inflamma- tory myofibroblastic tumor.N Engl J Med. 2010; 363(18):1727-1733.
  126. Mosse YP, Lim MS, Voss SD, et al. Safety and activity of crizotinib for paediatric pa- tients with refractory solid tumours or anaplastic large-cell lymphoma: a Children’s Oncology Group phase 1 consortium study. Lancet Oncol. 2013;14(6):472-480.
  127. Chavez C, Hoffman M. Complete remission of ALK-negative plasma cell granuloma (inflammatory myofibroblastic tumor) of the lung induced by celecoxib: a case report and review of the literature.Oncology Letter. 2013;5(5):1672-1676.
  128. Kusunoki-Nakamoto F, Marsukawa T, Tanaka M, et al. Successful treatment of an unresectable inflammatory myofibroblastic tumor of the frontal bone using a cyclo- oxygenase-2 inhibitor and methotrexate.Intern Med. 2013;52(5):623-628.
  129. Lee MH, Lee HB, Lee YC, et al. Bilateral multiple inflammatory myofibroblastic tumors of the lung successfully treated with corticosteroids.Lung. 2011;189(5):433-435.
  130. Tao YL, Wang AJ, Han JG, Wie P. Inflammatory myofibroblastic tumor successfully treated with chemotherapy and nonsteroidals: a case report.World J Gastroenterol. 2012;18(47):7100-7103.
  131. Penel N, Amela EY, Decanter G, Robin YM, Marec-Berard P. Solitary fibrous tumors and so-called hemangiopericytoma.Sarcoma. 2012;2012:690251.
  132. Levard A, Derbel O, Mèeus P, et al. Outcome of patients with advanced solitary fibrous tumors: the Centre Léon Bérard experience.BMC Cancer. 2013;13:109.
  133. Park MS, Ravi V, Conley A, et al. The role of chemotherapy in advanced solitary fi- brous tumors: a retrospective analysis.Clinical Sarcoma Research. 2013;3(1):7.
  134. Stacchiotti S, Tortoreto M, Bozzi F, et al. Dacarbazine in solitary fibrous tumor: a case series analysis and preclinical evidence vis-à-vis temozolomide and antiangiogenics.Clin Cancer Res;19(18):5192-5201.
  135. Parker MS, Patel SR, Ludwig JA, et al. Activity of temozolomide and bevacizumab in the treatment of locally advanced, recurrent, and metastatic hemangiopericytoma and malignant solitary fibrous tumor.Cancer. 2011;117(21):4939-4947.
  136. Edmonson JH, Ryan LM, Blum RH, et al. Randomized comparison of doxorubicin alone versus ifosfamide plus doxorubicin or mitmycin, doxorubicin, and cisplatin against advanced soft tissue sarcomas.J Clin Oncol. 1993;11(7):1269-1275.
  137. Le Cesne A, Antoine E, Speilmann M, et al. High-dose ifosfamide: circumvention of resistance to standard-dose ifosfamide in advanced soft tissue sarcomas.J Clin Oncol. (7):1600-1608.
  138. Spurrell EL, Fisher C, Thomas JM, Judson IR. Prognostic factors in advanced synovial sarcoma: an analysis of 104 patients treated at the Royal Marsden Hospital.Ann Oncol. 2005;16(3):437-444.
  139. Yasui H, Imura Y, Outani H, et al. Trabectedin is a promising antitumor agent for synovial sarcoma. J Chemother. 2016;28(5):417-424.
  140. U.S. food and drug administration grants breakthrough therapy designation for adaptimmune’s affinity enhanced t-cell therapy targeting ny-eso in synovial sarcoma [press release]. Oxford, UK: Adaptimmune Therapeutics plc; February 9, 2016. http://ir.adaptimmune.com/phoenix.zhtml?c=253991&p=irol-newsArticle&ID=2136858. Accessed January 9, 2017.
  141. Mackall C, D’Angelo S, Grupp S, et al. Open label non-randomized multi-cohort pilot study of genetically engineered NY-ESO-1 specific NY-ESO-1c259 SPEAR T-cellsTM in HLA-A*02+ patients with synovial sarcoma (NCT01343043). Presented at: 2016 ESMO Annual Congress; October 7-11, 2016; Copenhagen, Denmark. Abstract 1075P.
Related Videos
Related Content