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Reviews

Emerging therapies for adult soft tissue sarcoma

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References

  • Bannasch H, Eisenhardt SU, Grosu AL, et al. The diagnosis and treatment of soft tissue sarcomas of the limbs. Dtsch Arztebl Int 2011;108(3):32-8
  • Ducimetière F, Lurkin A, Ranchère-Vince D, et al. Incidence of sarcoma histotypes and molecular subtypes in a prospective epidemiological study with central pathology review and molecular testing. PLoS One 2011;6(8):e20294
  • Wibmer C, Leithner A, Zielonke N, et al. Increasing incidence rates of soft tissue sarcomas? A population-based epidemiologic study and literature review. Ann Oncol 2010;21(5):1106-11
  • Fletcher CDM, Bridge JA, Hogendoom PCW, Mertens F. WHO classification of tumours of soft tissue and bone. IARC press, Lyon, France; 2013
  • Burningham Z, Hashibe M, Spector L, Schiffman JD. The epidemiology of sarcoma. Clin Sarcoma Res 2012;2(1):14
  • Lahat G, Lazar A, Lev D. Sarcoma epidemiology and etiology: potential environmental and genetic factors. Surg Clin North Am 2008;88(3):451-81
  • Jain S, Xu R, Prieto VG, Lee P. Molecular classification of soft tissue sarcomas and its clinical applications. Int J Clin Exp Pathol 2010;3(4):416-28
  • Taylor BS, Barretina J, Maki RG, et al. Advances in sarcoma genomics and new therapeutic targets. Nat Rev Cancer 2011;11(8):541-57
  • Frith AE, Hirbe AC, Van Tine BA. Novel pathways and molecular targets for the treatment of sarcoma. Curr Oncol Rep 2013;15(4):378-85
  • Williamson SR, Bunde PJ, Montironi R, et al. Malignant perivascular epithelioid cell neoplasm (PEComa) of the urinary bladder with TFE3 gene rearrangement: clinicopathologic, immunohistochemical, and molecular features. Am J Surg Pathol 2013;37(10):1619-26
  • Martín Liberal J, Lagares-Tena L, Sáinz-Jaspeado M, et al. Targeted therapies in sarcomas: challenging the challenge. Sarcoma 2012;2012:626094
  • Quesada J, Amato R. The molecular biology of soft-tissue sarcomas and current trends in therapy. Sarcoma 2012;2012:849456
  • Demicco EG, Maki RG, Lev DC, Lazar AJ. New therapeutic targets in soft tissue sarcoma. Adv Anat Pathol 2012;19(3):170-80
  • Clark MA, Cyril F, Ian J, Meirion T. Soft-tissue sarcomas in adults. N Engl J Med 2005;353:701-11
  • Gronchi A, Olmi P, Casali PG. Combined modalities approach for localized adult extremity soft-tissue sarcoma. Expert Rev Anticancer Ther 2007;7(8):1135-44
  • Casali PG, Blay JY. Soft tissue sarcomas: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol 2010;21(Suppl 5):v198-203
  • Strauss DC, Hayes AJ, Thomas JM. Retroperitoneal tumours: review of management. Ann R Coll Surg Engl 2011;93(4):275-80
  • Gronchi A, Miceli R, Colombo C, et al. Frontline extended surgery is associated with improved survival in retroperitoneal low- to intermediate-grade soft tissue sarcomas. Ann Oncol 2012;23(4):1067-73
  • Colombo C, Randall RL, Andtbacka RH, Gronchi A. Surgery in soft tissue sarcoma: more conservative in extremities, more extended in the retroperitoneum. Expert Rev Anticancer Ther 2012;12(8):1079-87
  • Bonvalot S, Miceli R, Berselli M, et al. Aggressive surgery in retroperitoneal soft tissue sarcoma carried out at high-volume centers is safe and is associated with improved local control. Ann Surg Oncol 2010;17(6):1507-14
  • Strander H, Turesson I, Cavallin-Ståhl E. A systematic overview of radiation therapy effects in soft tissue sarcomas. Acta Oncol 2003;42(5-6):516-31
  • Yang JC, Chang AE, Baker AR, et al. Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 1998;16(1):197-203
  • Lee J, Park YJ, Yang DS, et al. Treatment outcome of conservative surgery plus postoperative radiotherapy for extremity soft tissue sarcoma. Radiat Oncol J 2012;30(2):62-9
  • Pisters PW, Harrison LB, Leung DH, et al. Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma. J Clin Oncol 1996;14(3):859-68
  • Fiore M, Grosso F, Lo Vullo S, et al. Myxoid/round cell and pleomorphic liposarcomas: prognostic factors and survival in a series of patients treated at a single institution. Cancer 2007;109(12):2522-31
  • Chung PW, Deheshi BM, Ferguson PC, et al. Radiosensitivity translates into excellent local control in extremity myxoid liposarcoma: a comparison with other soft tissue sarcomas. Cancer 2009;115(14):3254-61
  • 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):1191-7
  • Gronchi A, Verderio P, De Paoli A, et al. Quality of surgery and neoadjuvant combined therapy in the ISG-GEIS trial on soft tissue sarcomas of limbs and trunk wall. Ann Oncol 2013;24(3):817-23
  • Davis AM, O'Sullivan B, Bell RS, et al. Function and health status outcomes in a randomized trial comparing preoperative and postoperative radiotherapy in extremity soft tissue sarcoma. J Clin Oncol 2002;20(22):4472-7
  • Mohindra P, Neuman HB, Kozak KR. The role of radiation in retroperitoneal sarcomas. Curr Treat Options Oncol 2013. [Epub ahead of print]
  • Le Péchoux C, Musat E, Baey C, et al. Should adjuvant radiotherapy be administered in addition to front-line aggressive surgery (FAS) in patients with primary retroperitoneal sarcoma? Ann Oncol 2013;24(3):832-7
  • Roeder F, Schulz-Ertner D, Nikoghosyan AV, et al. A clinical phase I/II trial to investigate preoperative dose-escalated intensity-modulated radiation therapy (IMRT) and intraoperative radiation therapy (IORT) in patients with retroperitoneal soft tissue sarcoma. BMC Cancer 2012;12:287
  • Surgery with or without radiation therapy in treating patients with previously untreated nonmetastatic retroperitoneal soft tissue sarcoma (STRASS). 2013; ClinicalTrials.gov identifier: NCT01344018
  • Smith R, Demmy TL. Pulmonary metastasectomy for soft tissue sarcoma. Surg Oncol Clin N Am 2012;21(2):269-86
  • Kim S, Ott HC, Wright CD, et al. Pulmonary resection of metastatic sarcoma: prognostic factors associated with improved outcomes. Ann Thorac Surg 2011;92(5):1780-6; discussion 1786-7
  • Hatime M, Elmorabit B, Elkhoti Y, et al. Oligometastatic disease, a new concept: stereotactic irradiation for lung metastases. Literature review. Cancer Radiother 2012;16(5-6):351-7
  • Guadagnolo BA, Zagars GK, Ballo MT, et al. Mortality after cure of soft-tissue sarcoma treated with conservation surgery and radiotherapy. Cancer 2008;113(2):411-18
  • Billingsley KG, Burt ME, Jara E, et al. Pulmonary metastases from soft tissue sarcoma: analysis of patterns of diseases and postmetastasis survival. Ann Surg 1999;229(5):602-10
  • Ryan CW, Desai J. The past, present, and future of cytotoxic chemotherapy and pathway-directed targeted agents for soft tissue sarcoma. Am Soc Clin Oncol Educ Book 2013;386-93
  • 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):573-81
  • Woll PJ, Reichardt P, Le Cesne A, et al. Adjuvant chemotherapy with doxorubicin, ifosfamide, and lenograstim for resected soft-tissue sarcoma (EORTC 62931): a multicentre randomised controlled trial. Lancet Oncol 2012;13(10):1045-54
  • Frustaci S, Gherlinzoni F, De Paoli A, et al. Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian randomized cooperative trial. J Clin Oncol 2001;19(5):1238-47
  • Gronchi A, Frustaci S, Mercuri M, et al. Short, full-dose adjuvant chemotherapy in high-risk adult soft tissue sarcomas: a randomized clinical trial from the Italian Sarcoma Group and the Spanish Sarcoma Group. J Clin Oncol 2012;30(8):850-6
  • The ESMO/ European Sarcoma Network Working Group. Soft tissue and visceral sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 2012;23(Suppl 7):vii92-9
  • Kraybill WG, Harris J, Spiro IJ, et al. 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 2010;116(19):4613-21
  • Leahy M, Garcia Del Muro X, Reichardt P, et al. Chemotherapy treatment patterns and clinical outcomes in patients with metastatic soft tissue sarcoma. The sarcoma treatment and burden of illness in North America and Europe (SABINE) study. Ann Oncol 2012;23(10):2763-70
  • Karavasilis V, Seddon BM, Ashley S, et al. Significant clinical benefit of first-line palliative chemotherapy in advanced soft-tissue sarcoma: retrospective analysis and identification of prognostic factors in 488 patients. Cancer 2008;112(7):1585-91
  • Lee SH, Chang MH, Baek KK, et al. High-dose ifosfamide as second- or third-line chemotherapy in refractory bone and soft tissue sarcoma patients. Oncology 2011;80(3-4):257-61
  • Meazza C, Casanova M, Luksch R, et al. Prolonged 14-day continuous infusion of high-dose ifosfamide with an external portable pump: feasibility and efficacy in refractory pediatric sarcoma. Pediatr Blood Cancer 2010;55:617-20
  • Casali PG, Sanfilippo R, D'Incalci M. Trabectedin therapy for sarcomas. Curr Opin Oncol 2010;22(4):342-6
  • Demetri GD, Chawla SP, von Mehren M, et al. Efficacy and safety of trabectedin in patients with advanced or metastatic liposarcoma or leiomyosarcoma after failure of prior anthracyclines and ifosfamide: results of a randomized phase II study of two different schedules. J Clin Oncol 2009;27(25):4188-96
  • van Kesteren Ch, de Vooght MM, López-Lázaro L, et al. Yondelis (trabectedin, ET-743): the development of an anticancer agent of marine origin. Anticancer Drugs 2003;14(7):487-502
  • Gajdos C, Elias A. Trabectedin: safety and efficacy in the treatment of advanced sarcoma. Clin Med Insights Oncol 2011;5:35-43
  • Cassier PA, Dufresne A, Blay JY, Fayette J. Trabectedin and its potential in the treatment of soft tissue sarcoma. Ther Clin Risk Manag 2008;4(1):109-16
  • Hoiczyk M, Grabellus F, Podleska L, et al. Trabectedin in metastatic soft tissue sarcomas: role of pretreatment and age. Int J Oncol 2013;43(1):23-8
  • Fayette J, Coquard IR, Alberti L, et al. ET-743: a novel agent with activity in soft tissue sarcomas. Curr Opin Oncol 2005;10(10):827-32
  • Zucali PA, Bertuzzi A, Parra HJ, et al. The “old drug” dacarbazine as a second/third line chemotherapy in advanced soft tissue sarcomas. Invest New Drugs 2008;26(2):175-81
  • Gottlieb JA, Benjamin RS, Baker LH, et al. Role of DTIC (NSC-45388) in the chemotherapy of sarcomas. Cancer Treat Rep 1976;60(2):199-203
  • Buesa JM, Mouridsen HT, van Oosterom AT, et al. High-dose DTIC in advanced soft-tissue sarcomas in the adult. A phase II study of the E.O.R.T.C. Soft tissue and bone sarcoma group. Ann Oncol 1991;2(4):307-9
  • Lee HY, Shin SJ, Kim HS, et al. Weekly gemcitabine and docetaxel in refractory soft tissue sarcoma: a retrospective analysis. Cancer Res Treat 2012;44(1):43-9
  • Kaya AO, Büyükberber S, Ozkan M, et al. Efficacy and toxicity of gemcitabine plus docetaxel combination as a second line therapy for patients with advanced stage soft tissue sarcoma. Asian Pac J Cancer Prev 2012;13(2):463-7
  • Schöffski P, Ray-Coquard IL, Cioffi A, et al. Activity of eribulin mesylate in patients with soft-tissue sarcoma: a phase 2 study in four independent histological subtypes. Lancet Oncol 2011;12(11):1045-52
  • Phase 3 study to compare the efficacy and safety of eribulin with dacarbazine in subjects with soft tissue sarcoma. 2013; ClinicalTrials.gov identifier: NCT01327885
  • Leahy M, Ray-Coquard I, Verweij J, et al. Brostallicin, an agent with potential activity in metastatic soft tissue sarcoma: a phase II study from the European organisation for research and treatment of cancer soft tissue and bone sarcoma group. Eur J Cancer 2007;43(2):308-15
  • Randomized phase II study of Brostallicin or Doxorubicin as first line therapy in treating patients with relapsed, refracting or metastatic soft tissue sarcoma. 2010; ClinicalTrials.gov identifier: NCT00410462
  • Eriksson M. Histology-driven chemotherapy of soft-tissue sarcoma. Ann Oncol 2010;21(Suppl 7):vii270-6
  • Casali PG. Histology- and non-histology-driven therapy for treatment of soft tissue sarcomas. Ann Oncol 2012;23(Suppl 10):x167-9
  • Gronchi A, Casali PG. Adjuvant therapy for high-risk soft tissue sarcoma in the adult. Curr Treat Options Oncol 2013;14(3):415-24
  • Reichardt P, Lindner T, Pink D, et al. Chemotherapy in alveolar soft part sarcomas. What do we know? Eur J Cancer 2003;39(11):1511-16
  • Jones RL, Constantinidou A, Thway K, et al. Chemotherapy in clear cell sarcoma. Med Oncol 2011;28(3):859-63
  • Kontzoglou K, Stamatakos M, Polyzou E, et al. Dermatofibrosarcoma: a rare form of soft tissue. Management and review of the literature. Chirurgia (Bucur) 2011;106(5):653-6
  • Xing YM, Pan ZY, Li YW, et al. Diagnosis and treatment of epithelioid sarcoma. Zhonghua Zhong Liu Za Zhi 2011;33(11):872-4
  • Zehou O, Fabre E, Zelek L, et al. Chemotherapy for the treatment of malignant peripheral nerve sheath tumors in neurofibromatosis 1: a 10-year institutional review. Orphanet J Rare Dis 2013;8(1):127
  • Stacchiotti S, Libertini M, Negri T, et al. Response to chemotherapy of solitary fibrous tumour: a retrospective study. Eur J Cancer 2013;49(10):2376-83
  • Serrano C, George S. Leiomyosarcoma. Hematol Oncol Clin North Am 2013;27(5):957-74
  • Salah S, Yaser S, Salem A, et al. Factors influencing survival in metastatic synovial sarcoma: importance of patterns of metastases and the first-line chemotherapy regimen. Med Oncol 2013;30(3):639
  • 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
  • Grosso F, Sanfilippo R, Virdis E, et al. Trabectedin in myxoid liposarcomas (MLS): a long-term analysis of a single-institution series. Ann Oncol 2009;20(8):1439-44
  • Di Giandomenico S, Frapolli R, Bello E, et al. Mode of action of trabectedin in myxoid liposarcomas. Oncogene 2013. [Epub ahead of print]
  • Gronchi A, Bui BN, Bonvalot S, et al. Phase II clinical trial of neoadjuvant trabectedin in patients with advanced localized myxoid liposarcoma. Ann Oncol 2012;23(3):771-6
  • Localized high-risk soft tissue sarcomas of the extremities and trunk wall in adults: an integrating approach comprising standard vs histotype-tailored neoadjuvant chemotherapy. 2012; ClinicalTrials.gov identifier: NCT01710176
  • Monk BJ, Blessing JA, Street DG, et al. A phase II evaluation of trabectedin in the treatment of advanced, persistent, or recurrent uterine leiomyosarcoma: a gynecologic oncology group study. Gynecol Oncol 2012;124(1):48-52
  • Sanfilippo R, Grosso F, Jones RL, et al. Trabectedin in advanced uterine leiomyosarcomas: a retrospective case series analysis from two reference centers. Gynecol Oncol 2011;123(3):553-6
  • 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):1703-9
  • A study of trabectedin or dacarbazine for the treatment of patients with advanced liposarcoma or leiomyosarcoma. 2014; ClinicalTrials.gov identifier: NCT01343277
  • Chaigneau L, Kalbacher E, Thiery-Vuillemin A, et al. Efficacy of trabectedin in metastatic solitary fibrous tumor. Rare Tumors 2011;3(3):e29
  • Schuler MK, Richter S, Platzek I, et al. Trabectedin in the neoadjuvant treatment of high-grade pleomorphic sarcoma: report of a rare case and literature review. Case Rep Oncol Med 2013;2013:320797
  • Blay JY, von Mehren M, Samuels BL, et al. Phase I combination study of trabectedin and doxorubicin in patients with soft-tissue sarcoma. Clin Cancer Res 2008;14(20):6656-62
  • Pautier P, SelleFWeber BE, et al. A phase II single-arm multicenter study of doxorubicin in combination with trabectedin as a first-line treatment of advanced uterine leiomyosarcoma (U-LMS) and soft tissue LMS (ST-LMS): first results in patients with U-LMS. J Clin Oncol 2013;31(suppl): Abstract 10505
  • Svancárová L, Blay JY, Judson IR, et al. Gemcitabine in advanced adult soft-tissue sarcomas. A phase II study of the EORTC soft tissue and bone sarcoma group. Eur J Cancer 2002;38(4):556-9
  • Patel SR, Gandhi V, Jenkins J, et al. Phase II clinical investigation of gemcitabine in advanced soft tissue sarcomas and window evaluation of dose rate on gemcitabine triphosphate accumulation. J Clin Oncol 2001;19(15):3483-9
  • Bay JO, Ray-Coquard I, Fayette J, et al. Docetaxel and gemcitabine combination in 133 advanced soft-tissue sarcomas: a retrospective analysis. Int J Cancer 2006;119(3):706-11
  • 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 sarcomas: results of sarcoma alliance for research through collaboration study 002 [corrected]. J Clin Oncol 2007;25(19):2755-63
  • 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):1213-20
  • Schmitt T, Kosely F, Wuchter P, et al. Gemcitabine and docetaxel for metastatic soft tissue sarcoma - a single center experience. Onkologie 2013;36(7-8):415-20
  • Stacchiotti S, Palassini E, Sanfilippo R, et al. Gemcitabine in advanced angiosarcoma: a retrospective case series analysis from the Italian Rare Cancer Network. Ann Oncol 2012;23(2):501-8
  • A prospective randomised controlled phase III trial of gemcitabine and docetaxel compared with doxorubicin as fırst line treatment in previously untreated advance unresectable or metastatic soft tissue sarcomas. 2013; Available from: www.clinicaltrialsregister.eu/ctr-search/trial/2009-014907-29/GB [Last accessed 21 March 2013]
  • Sarcoma study of MORAb-004 utilization: research and clinical evaluation. 2013; ClinicalTrials.gov identifier: NCT01574716
  • 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):2433-6
  • Italiano A, Cioffi A, Penel N, et al. Comparison of doxorubicin and weekly paclitaxel efficacy in metastatic angiosarcomas. Cancer 2012;118(13):3330-6
  • 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):5269-74
  • Hirata T, Yonemori K, Ando M, et al. Efficacy of taxane regimens in patients with metastatic angiosarcoma. Eur J Dermatol 2011;21(4):539-45
  • García-Del-Muro X, López-Pousa A, Maurel J, et al. 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):2528-33
  • Stacchiotti S, Tortoreto M, Bozzi F, et al. Dacarbazine in solitary fibrous tumor: a case series analysis and preclinical evidence vis-a-vis temozolomide and antiangiogenics. Clin Cancer Res 2013;19(18):5192-201
  • Eilber FC, Dry SM. Diagnosis and management of synovial sarcoma. J Surg Oncol 2008;97(4):314-20
  • Canter RJ, Qin LX, Maki RG, et al. A synovial sarcoma-specific preoperative nomogram supports a survival benefit to ifosfamide-based chemotherapy and improves risk stratification for patients. Clin Cancer Res 2008;14(24):8191-7
  • Eilber FC, Brennan MF, Eilber FR, et al. Chemotherapy is associated with improved survival in adult patients with primary extremity synovial sarcoma. Ann Surg 2007;246(1):105-13
  • Roberta S, Rossella B, Andrea M, et al. Activity of high-dose continuous-infusione Ifosfamide in advanced well-differentiated/dedifferentiated liposarcoma. [Epub ahead to print]
  • Fujinami K, Kondoh K, Kondoh I, et al. A case of advanced retroperitoneal dedifferentiated liposarcoma treated effectively with high-dose ifosfamide. Hinyokika Kiyo 1999;45(7):463-6
  • Yokoi M, Hosokawa K, Funaki H, et al. A case of retroperitoneal dedifferentiated liposarcoma successfully treated with IFM and CDDP. Gan To Kagaku Ryoho 2009;36(12):2114-16
  • Carli M, Ferrari A, Mattke A, et al. Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group. J Clin Oncol 2005;23(33):8422-30
  • Cuneo KC, Riedel RF, Dodd LG, et al. Pathologic complete response of a malignant peripheral nerve sheath tumor in the lung treated with neoadjuvant Ifosfamide and radiation therapy. J Clin Oncol 2012;30(28):e291-3
  • Masui F, Yokoyama R, Soshi S, et al. A malignant peripheral nerve-sheath tumour responding to chemotherapy. J Bone Joint Surg Br 2004;86(1):113-15
  • Pápai Z, Bodoky G, Szántó J, et al. The efficacy of a combination of etoposide, ifosfamide, and cisplatin in the treatment of patients with soft tissue sarcoma. Cancer 2000;89(1):177-80
  • Kawai A, Chuman H, Makimoto A, et al. Ifosfamide - etoposide chemotherapy in patients with advanced adult soft tissue sarcomas (2004 ASCO Annual Meeting Proceedings (Post-Meeting Edition). J Clin Oncol 2004;22(14 Suppl):9062
  • Fiegl M, Schlemmer M, Wendtner CM, et al. Ifosfamide, carboplatin and etoposide (ICE) as second-line regimen alone and in combination with regional hyperthermia is active in chemo-pre-treated advanced soft tissue sarcoma of adults. Int J Hyperthermia 2004;20(6):661-70
  • Funakoshi Y, Kawai A, Hosono A, et al. A role of chemotherapy for malignant peripheral nerve sheath tumors (MPNSTs): A retrospective analysis of 40 cases in a single institution (2010 ASCO Annual Meeting Proceedings (Post-Meeting Edition). J Clin Oncol 2010;28(Suppl 15):e20512
  • Steins MB, Serve H, Zühlsdorf M, et al. Carboplatin/etoposide induces remission of metastasised malignant peripheral nerve tumours (malignant schwannoma) refractory to first-line therapy. Oncol Rep 2002;9(3):627-30
  • Kinebuchi Y, Noguchi W, Igawa Y, Nishizawa O. Recurrent retroperitoneal malignant nerve sheath tumor associated with neurofibromatosis type 1 responding to carboplatin and etoposide combined chemotherapy. Int J Clin Oncol 2005;10(5):353-6
  • Combination chemotherapy in treating patients with stage III or stage IV malignant peripheral nerve sheath tumors. 2013; ClinicalTrials.gov identifier: NCT00304083
  • Nuzhat H, Nidhi V. Curent concepts in pathology of soft tissue sarcoma. Indian J Surg Oncol 2011;2(4):302-8
  • Rauh-Hain JA, del Carmen MG. Endometrial stromal sarcoma: a systematic review. Obstet Gynecol 2013;122(3):676-83
  • Ioffe YJ, Li AJ, Walsh CS, et al. Hormone receptor expression in uterine sarcomas: prognostic and therapeutic roles. Gynecol Oncol 2009;115(3):466-71
  • Croom KF, Perry CM. Imatinib mesylate: in the treatment of gastrointestinal stromal tumours. Drugs 2003;63(5):513-22; discussion 523-4
  • Rutkowski P, Wozniak A, Switaj T. Advances in molecular characterization and targeted therapy in dermatofibrosarcoma protuberans. Sarcoma 2011;2011:959132
  • Kérob D, Porcher R, Vérola O, et al. Imatinib mesylate as a preoperative therapy in dermatofibrosarcoma: results of a multicenter phase II study on 25 patients. Clin Cancer Res 2010;16(12):3288-95
  • Rutkowski P, Van Glabbeke M, Rankin CJ, et al. Imatinib mesylate in advanced dermatofibrosarcoma protuberans: pooled analysis of two phase II clinical trials. J Clin Oncol 2010;28(10):1772-9
  • Stacchiotti S, Pedeutour F, Negri T, et al. Dermatofibrosarcoma protuberans-derived fibrosarcoma: clinical history, biological profile and sensitivity to imatinib. Int J Cancer 2011;129(7):1761-72
  • Blay JY, El Sayadi H, Thiesse P, et al. Complete response to imatinib in relapsing pigmented villonodular synovitis/tenosynovial giant cell tumor (PVNS/TGCT). Ann Oncol 2008;19(4):821-2
  • Cassier PA, Gelderblom H, Stacchiotti S, et al. Efficacy of imatinib mesylate for the treatment of locally advanced and/or metastatic tenosynovial giant cell tumor/pigmented villonodular synovitis. Cancer 2012;118(6):1649-55
  • Heudel P, Cassier P, Derbel O, et al. Pazopanib for the treatment of soft-tissue sarcoma. Clin Pharmacol 2012;4:65-70
  • Rajendra R, Jones RL, Pollack SM. Targeted treatment for advanced soft tissue sarcoma: profile of pazopanib. Onco Targets Ther 2013;6:217-22
  • Schöffski P. Pazopanib in the treatment of soft tissue sarcoma. Expert Rev Anticancer Ther 2012;12(6):711-23
  • Sleijfer S, Ray-Coquard I, Papai Z, et al. Pazopanib, a multikinase angiogenesis inhibitor, in patients with relapsed or refractory advanced soft tissue sarcoma: a phase II study from the European organisation for research and treatment of cancer-soft tissue and bone sarcoma group (EORTC study 62043). J Clin Oncol 2009;27(19):3126-32
  • van der Graaf WT, Blay JY, Chawla SP, et al. Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet 2012;379(9829):1879-86
  • Ranieri G, Mammì M, Donato Di Paola E, et al. Pazopanib a tyrosine kinase inhibitor with strong anti-angiogenetic activity: A new treatment for metastatic soft tissue sarcoma. Crit Rev Oncol Hematol 2014;89(2):322-9
  • Wilky BA, Meyer CF, Trent JC. Pazopanib in sarcomas: expanding the PALETTE. Curr Opin Oncol 2013;25(4):373-8
  • A trial comparing two medications as first treatment in elderly patients with metastatic or advanced soft tissue sarcoma (EPAZ). 2013; ClinicalTrials.gov identifier: NCT01861951
  • Papaetis GS, Syrigos KN. Sunitinib: a multitargeted receptor tyrosine kinase inhibitor in the era of molecular cancer therapies. BioDrugs 2009;23(6):377-89
  • George S, Merriam P, Maki RG, et al. Multicenter phase II trial of sunitinib in the treatment of nongastrointestinal stromal tumor sarcomas. J Clin Oncol 2009;27(19):3154-60
  • Stacchiotti S, Negri T, Libertini M, et al. Sunitinib malate in solitary fibrous tumor (SFT). Ann Oncol 2012;23(12):3171-9
  • Levard A, Derbel O, Méeus P, et al. Outcome of patients with advanced solitary fibrous tumors: the Centre Léon Bérard experience BMC Cancer. 2013;13:109
  • Stacchiotti S, Negri T, Zaffaroni N, et al. Sunitinib in advanced alveolar soft part sarcoma: evidence of a direct antitumor effect. Ann Oncol 22:1682-90.2011
  • Stacchiotti S, Tamborini E, Marrari A, et al. Response to sunitinib malate in advanced alveolar soft part sarcoma. Clin Cancer Res 2009;15(3):1096-104
  • Stacchiotti S, Dagrada GP, Morosi C, et al. Extraskeletal myxoid chondrosarcoma: tumor response to sunitinib. Clin Sarcoma Res 2012;2(1):22
  • Stacchiotti S, Grosso F, Palassini E, et al. Tumor response to sunitinib malate observed in clear-cell sarcoma. Ann Oncol 2010;21(5):1130-1
  • Italiano A, Kind M, Cioffi A, et al. Clinical activity of sunitinib in patients with advanced desmoplastic round cell tumor: a case series. Target Oncol 2013;8(3):211-13
  • Jakob J, Rauch G, Wenz F, Hohenberger P. Phase I trial of concurrent sunitinib and radiation therapy as preoperative treatment for soft tissue sarcoma. BMJ Open 2013;3(9):e003626
  • Sahade M, Caparelli F, Hoff PM. Cediranib: a VEGF receptor tyrosine kinase inhibitor. Future Oncol 2012;8(7):775-81
  • Kummar S, Allen D, Monks A, et al. Cediranib for metastatic alveolar soft part sarcoma. J Clin Oncol 2013;31(18):2296-302
  • Rubina K, Talovskaya E, Cherenkov V, et al. LDL induces intracellular signalling and cell migration via atypical LDL-binding protein T-cadherin. Mol Cell Biochem 2005;273(1-2):33-41
  • Rini BI. Sorafenib. Expert Opin.Pharmacother 2006;7(4):453-61
  • Escudier B, Eisen T, Stadler WM, et al. Sorafenib in advanced clear-cell renal-cell carcinoma. N Engl J Med 2007;356(2):125-34
  • Llovet JM, Ricci S, Mazzaferro V, et al. Sorafenib in advanced hepatocellular carcinoma. N Engl J Med 2008;359(4):378-90
  • 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):3133-40
  • Ray-Coquard I, Italiano A, Bompas E, et al. Sorafenib for patients with advanced angiosarcoma: a phase II Trial from the French Sarcoma Group (GSF/GETO). Oncologist 2012;17(2):260-6
  • von Mehren M, Rankin C, Goldblum JR, et al. Phase 2 Southwest Oncology Group-directed intergroup trial (S0505) of sorafenib in advanced soft tissue sarcomas. Cancer 2012;118(3):770-6
  • Valentin T, Fournier C, Penel N, et al. Sorafenib in patients with progressive malignant solitary fibrous tumors: a subgroup analysis from a phase II study of the French Sarcoma Group (GSF/GETO). Invest New Drugs 2013;31(6):1626-7
  • Kamar FG, Kairouz VF, Sabri AN. Dermatofibrosarcoma protuberans (DFSP) successfully treated with sorafenib: case report. Clin Sarcoma Res 2013;3(1):5
  • Mir O, Boudou-Rouquette P, Larousserie F, et al. Objective response to sorafenib in advanced clear-cell sarcoma. Ann Oncol 2012;23(3):807-9
  • Basso U, Brunello A, Bertuzzi A, Santoro A. Sorafenib is active on lung metastases from synovial sarcoma. Ann Oncol 2009;20(2):386-7
  • Amit L, Ben-Aharon I, Vidal L, et al. The impact of Bevacizumab (Avastin) on survival in metastatic solid tumors--a meta-analysis and systematic review. PLoS One 2013;8(1):e51780
  • 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):7135-42
  • Yang P, Zhu Q, Jiang F. Combination therapy for scalp angiosarcoma using bevacizumab and chemotherapy: a case report and review of literature. Chin J Cancer Res 2013;25(3):358-61
  • 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):257-63
  • Yoon SS, Duda DG, Karl DL, et al. Phase II study of neoadjuvant bevacizumab and radiotherapy for resectable soft tissue sarcomas. Int J Radiat Oncol Biol Phys 2011;81(4):1081-90
  • Mir O, Boudou-Rouquette P, Larousserie F, et al. Durable clinical activity of single-agent bevacizumab in a nonagenarian patient with metastatic alveolar soft part sarcoma. Anticancer Drugs 2012;23(7):745-8
  • Park 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):4939-47
  • Agulnik M. New developments in mammalian target of rapamycin inhibitors for the treatment of sarcoma. Cancer 2012;118(6):1486-97
  • Blay JY. Updating progress in sarcoma therapy with mTOR inhibitors. Ann Oncol 2011;22(2):280-7
  • Morath C, Arns W, Schwenger V, et al. Sirolimus in renal transplantation. Nephrol Dial Transplant 2007;22(Suppl 8):viii61-5
  • Ito D, Fujimoto K, Mori T, et al. In vivo antitumor effect of the mTOR inhibitor CCI-779 and gemcitabine in xenograft models of human pancreatic cancer. Int J Cancer 2006;118(9):2337-43
  • Zhao YM, Zhou Q, Xu Y, et al. Antiproliferative effect of rapamycin on human T-cell leukemia cell line Jurkat by cell cycle arrest and telomerase inhibition. Acta Pharmacol Sin 2008;29(4):481-8
  • Bissler JJ, McCormack FX, Young LR, et al. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. N Engl J Med 2008;358(2):140-51
  • Sanfilippo R, Dei Tos AP, Casali PG. Myxoid liposarcoma and the mammalian target of rapamycin pathway. Curr Opin Oncol 2013;25(4):379-83
  • Merimsky O, Bernstein-Molho R, Sagi-Eisenberg R. Targeting the mammalian target of rapamycin in myxoid chondrosarcoma. Anticancer Drugs 2008;19(10):1019-21
  • Hohman DW, Noghrehkar D, Ratnayake S. Lymphangioleiomyomatosis: a review. Eur J Intern Med 2008;19(5):319-24
  • 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):835-40
  • Pan CC, Chung MY, Ng KF, et al. Constant allelic alteration on chromosome 16p (TSC2 gene) in perivascular epithelioid cell tumour (PEComa): genetic evidence for the relationship of PEComa with angiomyolipoma. J Pathol 2008;214(3):387-93
  • Motzer RJ, Escudier B, Oudard S, et al. Efficacy of everolimus in advanced renal cell carcinoma: a double-blind, randomised, placebo-controlled phase III trial. Lancet 2008;372(9637):449-56
  • Yoo C, Lee J, Rha SY, et al. Multicenter phase II study of everolimus in patients with metastatic or recurrent bone and soft-tissue sarcomas after failure of anthracycline and ifosfamide. Invest New Drugs 2013;31(6):1602-8
  • Mita MM, Gong J, Chawla SP. Ridaforolimus in advanced or metastatic soft tissue and bone sarcomas. Expert Rev Clin Pharmacol 2013;6(5):465-82
  • Spreafico A, Mackay HJ. Current Phase II clinical data for ridaforolimus in cancer. Expert Opin Investig Drugs 2013;22(11):1485-93
  • Mita M, Sankhala K, Abdel-Karim I, et al. Deforolimus (AP23573) a novel mTOR inhibitor in clinical development. Expert Opin Investig Drugs 2008;17(12):1947-54
  • Chawla SP, Staddon AP, Baker LH, et al. Phase II study of the mammalian target of rapamycin inhibitor ridaforolimus in patients with advanced bone and soft tissue sarcomas. J Clin Oncol 2012;30(1):78-84
  • Demetri GD, Chawla SP, Ray-Coquard I, et al. Results of an international randomized phase III trial of the mammalian target of rapamycin inhibitor ridaforolimus versus placebo to control metastatic sarcomas in patients after benefit from prior chemotherapy. J Clin Oncol 2013;31(19):2485-92
  • Kurzrock R, Patnaik A, Aisner J, et al. A phase I study of weekly R1507, a human monoclonal antibody insulin-like growth factor-I receptor antagonist, in patients with advanced solid tumors. Clin Cancer Res 2010;16(8):2458-65
  • O'Neill A, Shah N, Zitomersky N, et al. Insulin-like growth factor 1 receptor as a therapeutic target in ewing sarcoma: lack of consistent upregulation or recurrent mutation and a review of the clinical trial literature. Sarcoma 2013;2013:450478
  • Sun Y, Gao D, Liu Y, et al. IGF2 is critical for tumorigenesis by synovial sarcoma oncoprotein SYT-SSX1. Oncogene 2006;25(7):1042-52
  • Olmos D, Postel-Vinay S, Molife LR, et al. Safety, pharmacokinetics, and preliminary activity of the anti-IGF-1R antibody figitumumab (CP-751,871) in patients with sarcoma and Ewing's sarcoma: a phase 1 expansion cohort study. Lancet Oncol 2010;11(2):129-35
  • Quek R, Wang Q, Morgan JA, et al. Combination mTOR and IGF-1R inhibition: phase I trial of everolimus and figitumumab in patients with advanced sarcomas and other solid tumors. Clin Cancer Res 2011;17(4):871-9
  • Stacchiotti S, Negri T, Palassini E, et al. Sunitinib malate and figitumumab in solitary fibrous tumor: patterns and molecular bases of tumor response. Mol Cancer Ther 2010;9(5):1286-97
  • Ma X, Ezzeldin HH, Diasio RB. Histone deacetylase inhibitors: current status and overview of recent clinical trials. Drugs 2009;69(14):1911-34
  • Lane AA, Chabner BA. Histone deacetylase inhibitors in cancer therapy. J Clin Oncol 2009;27(32):5459-68
  • Mann BS, Johnson JR, Cohen MH, et al. FDA approval summary: vorinostat for treatment of advanced primary cutaneous T-cell lymphoma. Oncologist 2007;12(10):1247-52
  • Ito T, Ouchida M, Morimoto Y, et al. Significant growth suppression of synovial sarcomas by the histone deacetylase inhibitor FK228 in vitro and in vivo. Cancer Lett 2005;224(2):311-19
  • Sakimura R, Tanaka K, Nakatani F, et al. Antitumor effects of histone deacetylase inhibitor on Ewing's family tumors. Int J Cancer 2005;116(5):784-92
  • Hrzenjak A, Moinfar F, Kremser ML, et al. Histone deacetylase inhibitor vorinostat suppresses the growth of uterine sarcomas in vitro and in vivo. Mol Cancer 2010;9:49
  • Cassier PA, Lefranc A, Y Amela E, et al. A phase II trial of panobinostat in patients with advanced pretreated soft tissue sarcoma. A study from the French Sarcoma Group. Br J Cancer 2013;109(4):909-14
  • Levard A, Tassy L, Cassier PA. Emerging therapies for soft-tissue sarcomas. Hematol Oncol Clin North Am 2013;27(5):1063-78
  • Roma J, Almazán-Moga A, Sánchez de Toledo J, Gallego S. Notch, wnt, and hedgehog pathways in rhabdomyosarcoma: from single pathways to an integrated network. Sarcoma 2012;2012:695603
  • Kelleher FC, Cain JE, Healy JM, et al. Prevailing importance of the hedgehog signaling pathway and the potential for treatment advancement in sarcoma. Pharmacol Ther 2012;136(2):153-68
  • Liu H, Gu D, Xie J. Clinical implications of hedgehog signaling pathway inhibitors. Chin J Cancer 2011;30(1):13-26
  • Tolcher AW, Messersmith WA, Mikulski SM, et al. Phase I study of RO4929097, a gamma secretase inhibitor of Notch signaling, in patients with refractory metastatic or locally advanced solid tumors. J Clin Oncol 2012;30(19):2348-53
  • Wang CY, Wei Q, Han I, et al. Hedgehog and Notch signaling regulate self-renewal of undifferentiated pleomorphic sarcomas. Cancer Res 2012;72(4):1013-22
  • Binh MB, Sastre-Garau X, Guillou L, et al. MDM2 and CDK4 immunostainings are useful adjuncts in diagnosing well-differentiated and dedifferentiated liposarcoma subtypes: a comparative analysis of 559 soft tissue neoplasms with genetic data. Am J Surg Pathol 2005;29(10):1340-7
  • Aleixo PB, Hartmann AA, Menezes IC, et al. Can MDM2 and CDK4 make the diagnosis of well differentiated/dedifferentiated liposarcoma? An immunohistochemical study on 129 soft tissue tumours. J Clin Pathol 2009;62(12):1127-35
  • Ray-Coquard I, Blay JY, Italiano A, et al. Effect of the MDM2 antagonist RG7112 on the P53 pathway in patients with MDM2-amplified, well-differentiated or dedifferentiated liposarcoma: an exploratory proof-of-mechanism study. Lancet Oncol 2012;13(11):1133-40
  • 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):2024-8
  • Shaw AT, Kim DW, Nakagawa K, et al. Crizotinib versus chemotherapy in advanced ALK-positive lung cancer. N Engl J Med 2013;368(25):2385-94
  • Butrynski JE, D'Adamo DR, Hornick JL, et al. Crizotinib in ALK-rearranged inflammatory myofibroblastic tumor. N Engl J Med 2010;363(18):1727-33
  • Cross-tumoral Phase 2 clinical trial exploring crizotinib (PF-02341066) in patients with advanced tumors induced by causal alterations of ALK and/or MET (“CREATE”). 2013; ClinicalTrials.gov identifier: NCT01524926
  • Dunn GP, Old LJ, Schreiber RD. The immunobiology of cancer immunosurveillance and immunoediting. Immunity 2004;21(2):137-48
  • Kudchadkar RR, Gonzalez R, Lewis K. New targeted therapies in melanoma. Cancer Contr 2013;20(4):282-8
  • Lynch TJ, Bondarenko I, Luft A, et al. Ipilimumab in combination with paclitaxel and carboplatin as first-line treatment in stage IIIB/IV non-small-cell lung cancer: results from a randomized, double-blind, multicenter phase II study. J Clin Oncol 2012;30(17):2046-54
  • Brahmer JR, Drake CG, Wollner I, et al. Phase I study of single-agent anti-programmed death-1 (MDX-1106) in refractory solid tumors: safety, clinical activity, pharmacodynamics, and immunologic correlates. J Clin Oncol 2010;28(19):3167-75
  • Brahmer JR, Tykodi SS, Chow LQ, et al. Safety and activity of anti-PD-L1 antibody in patients with advanced cancer. N Engl J Med 2012;366(26):2455-65
  • Robbins PF, Morgan RA, Feldman SA, et al. Tumor regression in patients with metastatic synovial cell sarcoma and melanoma using genetically engineered lymphocytes reactive with NY-ESO-1. J Clin Oncol 2011;29(7):917-24

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