Options for treating different soft tissue sarcoma subtypes

References

• 1 Mastrangelo G, Coindre JM, Ducimetière F et al. Incidence of soft tissue sarcoma and beyond: a population-based prospective study in three European regions. Cancer 118(21), 5339–5348 (2012).
  MedlineGoogle Scholar
• 2 Fletcher CDM, Bridge JA, Hogendoorn PCW, Bridge J. WHO Classification of Tumours of Soft Tissue and Bone (4th Edition). IARC Press, Lyon, France (2013).
  Google Scholar
• 3 ESMO/European Sarcoma Network Working Group. Soft tissue and visceral sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann. Oncol. 25(Suppl. 3), iii102–iii112 (2014).
  MedlineGoogle Scholar
• 4 Reichardt P. Soft tissue sarcomas, a look into the future: different treatments for different subtypes. Future Oncol. 10(8 Suppl.), s19–s27 (2014).
  CASGoogle Scholar
• 5 Ricci S, Stone RL, Fader AN. Uterine leiomyosarcoma: epidemiology, contemporary treatment strategies and the impact of uterine morcellation. Gynecol. Oncol. 145(1), 208–216 (2017).
  MedlineGoogle Scholar
• 6 Miller H, Ike C, Parma J, Masand RP, Mach CM, Anderson ML. Molecular targets and emerging therapeutic options for uterine leiomyosarcoma. Sarcoma 2016, 7018106 (2016).
  MedlineGoogle Scholar
• 7 Raut CP, Nucci MR, Wang Q et al. Predictive value of FIGO and AJCC staging systems in patients with uterine leiomyosarcoma. Eur. J. Cancer 45(16), 2818–2824 (2009).
  MedlineGoogle Scholar
• 8 George S, Barysauskas C, Serrano C et al. Retrospective cohort study evaluating the impact of intraperitoneal morcellation on outcomes of localized uterine leiomyosarcoma. Cancer 120(20), 3154–3158 (2014).
  MedlineGoogle Scholar
• 9 Raspagliesi F, Maltese G, Bogani G et al. Morcellation worsens survival outcomes in patients with undiagnosed uterine leiomyosarcomas: a retrospective MITO group study. Gynecol. Oncol. 144(1), 90–95 (2017).
  MedlineGoogle Scholar
• 10 Takamizawa S, Minakami H, Usui R et al. Risk of complications and uterine malignancies in women undergoing hysterectomy for presumed benign leiomyomas. Gynecol. Obstet. Invest. 48(3), 193–196 (1999).
  Medline CASGoogle Scholar
• 11 Hensley ML, Barrette BA, Baumann K et al. Gynecologic Cancer InterGroup (GCIG) consensus review: uterine and ovarian leiomyosarcomas. Int. J. Gynecol. Cancer 24(9 Suppl. 3), S61–S66 (2014).
  MedlineGoogle Scholar
• 12 Dangoor A, Seddon B, Gerrand C, Grimer R, Whelan J, Judson I. UK guidelines for the management of soft tissue sarcomas. Clin. Sarcoma Res. 6, 20 (2016).
  MedlineGoogle Scholar
• 13 Reed NS, Mangioni C, Malmström H et al. Phase III randomized study to evaluate the role of adjuvant pelvic radiotherapy in the treatment of uterine sarcomas stages I and II: an European Organization for Research and Treatment of Cancer Gynaecological Cancer Group Study (protocol 55874). Eur. J. Cancer 44(6), 808–818 (2008).
  Medline CASGoogle Scholar
• 14 O'Cearbhaill R, Hensley ML. Optimal management of uterine leiomyosarcoma. Expert Rev. Anticancer Ther. 10(2), 153–169 (2010).
  MedlineGoogle Scholar
• 15 Hensley ML, Wathen JK, Maki RG et al. Adjuvant therapy for high-grade, uterus-limited leiomyosarcoma: results of a Phase II trial (SARC 005). Cancer 119(8), 1555–1561 (2013).
  Medline CASGoogle Scholar
• 16 Clinical Trial database: NCT01533207. https://clinicaltrials.gov/ct2/show/NCT01533207.
  Google Scholar
• 17 Pautier P, Floquet A, Gladieff L et al. A randomized clinical trial of adjuvant chemotherapy with doxorubicin, ifosfamide and cisplatin followed by radiotherapy versus radiotherapy alone in patients with localized uterine sarcomas (SARCGYN study). A study of the French Sarcoma Group. Ann. Oncol. 24(4), 1099–1104 (2013).
  Medline CASGoogle Scholar
• 18 Ray-Coquard I, Rizzo E, Blay JY et al. Impact of chemotherapy in uterine sarcoma (UtS): review of 13 clinical trials from the EORTC Soft Tissue and Bone Sarcoma Group (STBSG) involving advanced/metastatic UtS compared to other soft tissue sarcoma (STS) patients treated with first line chemotherapy. Gynecol. Oncol. 142(1), 95–101 (2016).
  Medline CASGoogle Scholar
• 19 NCCN Clinical Practice Guidelines in Oncology. Uterine neoplasms. Version 2.2016. National Comprehensive Cancer Network. www.nccn.org/professionals/physician_gls/f_guidelines.asp.
  Google Scholar
• 20 George S, Feng Y, Manola J et al. Phase II trial of aromatase inhibition with letrozole in patients with uterine leiomyosarcomas expressing estrogen and/or progesterone receptors. Cancer 120(5), 738–743 (2014).
  Medline CASGoogle Scholar
• 21 Sleijfer S, Ouali M, van Glabbeke M et al. Prognostic and predictive factors for outcome to first-line ifosfamide-containing chemotherapy for adult patients with advanced soft tissue sarcomas: an exploratory, retrospective analysis on large series from the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group (EORTC-STBSG). Eur. J. Cancer 46(1), 72–83 (2010).
  Medline CASGoogle Scholar
• 22 Ray-Coquard IL, Sleijfer S, Litière S et al. Pazopanib in uterine sarcoma (UtS): review of two European Organization for Research and Treatment of Cancer (EORTC) and GSK clinical trials 62043 and 62072 on pazopanib for soft tissue sarcoma (STS). J. Clin. Oncol. 32(Suppl), Abstract 10579 (2014).
  Google Scholar
• 23 Hensley ML, Blessing JA, Degeest K, Abulafia O, Rose PG, Homesley HD. Fixed-dose rate gemcitabine plus docetaxel as second-line therapy for metastatic uterine leiomyosarcoma: a Gynecologic Oncology Group Phase II study. Gynecol. Oncol. 109(3), 323–328 (2008).
  Medline CASGoogle Scholar
• 24 Demetri GD, von Mehren M, Jones RL et al. 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(8), 786–793 (2016).
  Medline CASGoogle Scholar
• 25 Sanfilippo R, Grosso F, Jones RL et al. Trabectedin in advanced uterine leiomyosarcomas: a retrospective case series analysis from two reference centers. Gynecol. Oncol. 123(3), 553–556 (2011).
  Medline CASGoogle Scholar
• 26 Amant F, Lorusso D, Mustea A, Duffaud F, Pautier P. Management strategies in advanced uterine leiomyosarcoma: focus on trabectedin. Sarcoma 2015, 704124 (2015).
  MedlineGoogle Scholar
• 27 Grignani G, Martín-Broto J, Schuler M, Reichardt P. Trabectedin clinical cases: use according to indication in diverse clinical scenarios. Future Oncol. 11(11 Suppl.), 15–24 (2015).
  CASGoogle Scholar
• 28 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 6(8), e20294 (2011).
  Medline CASGoogle Scholar
• 29 Crago AM, Singer S. Clinical and molecular approaches to well differentiated and dedifferentiated liposarcoma. Curr. Opin. Oncol. 23(4), 373–378 (2011).
  Medline CASGoogle Scholar
• 30 Saponara M, Stacchiotti S, Gronchi A. Pharmacological therapies for liposarcoma. Expert Rev. Clin. Pharmacol. 10(4), 361–377 (2017).
  Medline CASGoogle Scholar
• 31 Schwab JH, Boland P, Guo T et al. Skeletal metastases in myxoid liposarcoma: an unusual pattern of distant spread. Ann. Surg. Oncol. 14(4), 1507–1514 (2007).
  MedlineGoogle Scholar
• 32 Dei Tos AP. Liposarcomas: diagnostic pitfalls and new insights. Histopathology 64(1), 38–52 (2014).
  MedlineGoogle Scholar
• 33 Ghadimi MP, Liu P, Peng T et al. Pleomorphic liposarcoma: clinical observations and molecular variables. Cancer 117(23), 5359–5369 (2011).
  Medline CASGoogle Scholar
• 34 Thway K, Jones RL, Noujaim J, Zaidi S, Miah AB, Fisher C. Dedifferentiated liposarcoma: updates on morphology, genetics and therapeutic strategies. Adv. Anat. Pathol. 23(1), 30–40 (2016).
  Medline CASGoogle Scholar
• 35 Tseng WW, Somaiah N, Lazar AJ, Lev DC, Pollock RE. Novel systemic therapies in advanced liposarcoma: a review of recent clinical trial results. Cancers (Basel) 5(2), 529–549 (2013).
  MedlineGoogle Scholar
• 36 Le Cesne A, Ray-Coquard I, Duffaud F et al. Trabectedin in patients with advanced soft tissue sarcoma: a retrospective national analysis of the French Sarcoma Group. Eur. J. Cancer 51(6), 742–750 (2015).
  Medline CASGoogle Scholar
• 37 Blay JY, Italiano A, Ray-Coquard I et al. Long-term outcome and effect of maintenance therapy in patients with advanced sarcoma treated with trabectedin: an analysis of 181 patients of the French ATU compassionate use program. BMC Cancer 13, 64 (2013).
  Medline CASGoogle Scholar
• 38 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. 24(6), 1703–1709 (2013).
  Medline CASGoogle Scholar
• 39 Grosso F, Jones RL, Demetri GD et al. Efficacy of trabectedin (ecteinascidin-743) in advanced pretreated myxoid liposarcomas: a retrospective study. Lancet Oncol. 8(7), 595–602 (2007).
  Medline CASGoogle Scholar
• 40 Kus T, Aktas G, Kalender ME, Tutar E, Ulker E, Camci C. Complete response of a recurrent-metastatic liposarcoma with dedifferentiated histological features following the administration of trabectedin and review of literature. J. Cancer Res. Ther. 11(4), 974–976 (2015).
  Medline CASGoogle Scholar
• 41 Martín-Broto J, Le Cesne A, Reichardt P. The importance of treating by histological subtype in advanced soft tissue sarcoma. Future Oncol. 13(1s), 23–31 (2017).
  CASGoogle Scholar
• 42 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 organization for research and treatment of cancer-soft tissue and bone sarcoma group (EORTC study 62043). J. Clin. Oncol. 27(19), 3126–3132 (2009).
  Medline CASGoogle Scholar
• 43 Valverde CM, Martín-Broto J, Lopez-Martin JA et al. Phase II clinical trial evaluating the activity and tolerability of pazopanib in patients (pts) with advanced and/or metastatic liposarcoma (LPS): a joint Spanish Sarcoma Group (GEIS) and German Interdisciplinary Sarcoma Group (GISG) Study – NCT01692496. J. Clin. Oncol. 34(Suppl.), Abstract 11039 (2016).
  Google Scholar
• 44 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. 25(19), 2755–2563 (2007).
  Medline CASGoogle Scholar
• 45 Chawla SP, Schöffski P, Grignani G et al. Subtype-specific activity in liposarcoma (LPS) patients (pts) from a Phase III, open-label, randomized study of eribulin (ERI) versus dacarbazine (DTIC) in pts with advanced LPS and leiomyosarcoma (LMS). J. Clin. Oncol 34(Suppl.), Abstract 11037 (2016).
  MedlineGoogle Scholar
• 46 Van Glabbeke M, Verweij J, Judson I, Nielsen OS, EORTC Soft Tissue and Bone Sarcoma Group. Progression-free rate as the principal end-point for phase II trials in soft-tissue sarcomas. Eur. J. Cancer 38(4), 543–549 (2002).
  Medline CASGoogle Scholar
• 47 Gounaris I, Hatcher HM, Davidson D et al. Trabectedin for advanced soft tissue sarcomas: a single institution experience. Future Oncol. 10(11), 1843–1851 (2014).
  CASGoogle Scholar
• 48 Mertens F, Antonescu CR, Hohenberger P et al. Translocation-related sarcomas. Semin. Oncol. 36(4), 312–323 (2009).
  Medline CASGoogle Scholar
• 49 Kawai A, Araki N, Sugiura H et al. Trabectedin monotherapy after standard chemotherapy versus best supportive care in patients with advanced, translocation-related sarcoma: a randomized, open-label, Phase II study. Lancet Oncol. 16(4), 406–416 (2015).
  Medline CASGoogle Scholar
• 50 Nielsen TO, Poulin NM, Ladanyi M. Synovial sarcoma: recent discoveries as a roadmap to new avenues for therapy. Cancer Discov. 5(2), 124–134 (2015).
  Medline CASGoogle Scholar
• 51 Kubo T, Shimose S, Fujimori J, Furuta T, Ochi M. Prognostic value of SS18-SSX fusion type in synovial sarcoma; systematic review and meta-analysis. Springerplus 4, 375 (2015).
  MedlineGoogle Scholar
• 52 Martín-Broto J. Goal 3: controlling tumor growth and preserving quality of life. Cancer Chemother. Rev. 11(Suppl. 5), 13–16 (2016).
  Google Scholar
• 53 Clinical Trial Database: NCT02275286. https://clinicaltrials.gov/ct2/show/NCT02275286.
  Google Scholar
• 54 Gronchi A, Hindi N, Cruz J et al. Trabectedin and radiotherapy in soft-tissue sarcoma (TRASTS) study: an international, prospective, Phase I/II trial – a collaborative Spanish (GEIS), Italian (ISG) and French (FSG) groups study. J. Clin. Oncol. 33(Suppl.), Abstract 11061 (2017).
  Google Scholar
• 55 Dagrada GP, Spagnuolo RD, Mauro V et al. Solitary fibrous tumors: loss of chimeric protein expression and genomic instability mark dedifferentiation. Mod. Pathol. 28(8), 1074–1083 (2015).
  Medline CASGoogle Scholar
• 56 Park MS, Ravi V, Conley A et al. The role of chemotherapy in advanced solitary fibrous tumors: a retrospective analysis. Clin. Sarcoma Res. 3(1), 7 (2013).
  Medline CASGoogle Scholar
• 57 Chmielecki J, Crago AM, Rosenberg M et al. Whole-exome sequencing identifies a recurrent NAB2-STAT6 fusion in solitary fibrous tumors. Nat. Genet. 45(2), 131–132 (2013).
  Medline CASGoogle Scholar
• 58 Clinical Trials Database: NCT02066285. https://clinicaltrials.gov/ct2/show/NCT02066285.
  Google Scholar
• 59 Stacchiotti S, Libertini M, Negri T et al. Response to chemotherapy of solitary fibrous tumour: a retrospective study. Eur. J. Cancer 49(10), 2376–2383 (2013).
  Medline CASGoogle Scholar
• 60 Bylicki O, Rouvière D, Cassier P et al. Assessing the multimodal management of advanced solitary fibrous tumors of the pleura in a routine practice setting. J. Thorac. Oncol. 10(2), 309–315 (2015).
  MedlineGoogle Scholar
• 61 Stacchiotti S, Saponara M, Frapolli R et al. Patient-derived solitary fibrous tumour xenografts predict high sensitivity to doxorubicin/dacarbazine combination confirmed in the clinic and highlight the potential effectiveness of trabectedin or eribulin against this tumour. Eur. J. Cancer 76, 84–92 (2017).
  Medline CASGoogle Scholar
• 62 Khalifa J, Ouali M, Chaltiel L et al. Efficacy of trabectedin in malignant solitary fibrous tumors: a retrospective analysis from the French Sarcoma Group. BMC Cancer 15, 700 (2015).
  Medline CASGoogle Scholar
• 63 Le Cesne A, Ray-Coquard I, Duffaud F et al. Trabectedin in patients with advanced soft tissue sarcoma: a retrospective national analysis of the French Sarcoma Group. Eur. J. Cancer 51(6), 742–750 (2015).
  Medline CASGoogle Scholar
• 64 Clinical Trials Database: NCT03023124. https://clinicaltrials.gov/ct2/show/NCT03023124.
  Google Scholar
• 65 Maruzzo M, Martin-Liberal J, Messiou C et al. Pazopanib as first line treatment for solitary fibrous tumours: the Royal Marsden Hospital experience. Clin. Sarcoma Res. 5, 5 (2015).
  Medline CASGoogle Scholar
• 66 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 117(21), 4939–4497 (2011).
  Medline CASGoogle Scholar
• 67 Stacchiotti S, Negri T, Libertini M et al. Sunitinib malate in solitary fibrous tumor (SFT). Ann. Oncol. 23(12), 3171–3179 (2012).
  Medline CASGoogle Scholar
• 68 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 31(6), 1626–1627 (2013).
  Medline CASGoogle Scholar
• 69 Pervaiz N, Colterjohn N, Farrokhyar F, Tozer R, Figueredo A, Ghert M. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer 113(3), 573–581 (2008).
  MedlineGoogle Scholar
• 70 Gronchi A, Ferrari S, Quagliuolo V et al. Histotype-tailored neoadjuvant chemotherapy versus standard chemotherapy in patients with high-risk soft-tissue sarcomas (ISG-STS 1001): an international, open-label, randomized, controlled, Phase III, multicenter trial. Lancet Oncol. 18(6), 812–822 (2017).
  Medline CASGoogle Scholar
• 71 Movva S, von Mehren M, Ross EA, Handorf E. Patterns of chemotherapy administration in high-risk soft tissue sarcoma and impact on overall survival. J. Natl Compr. Canc. Netw. 13(11), 1366–1374 (2015).
  Medline CASGoogle Scholar
• 72 Tawbi HA, 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. 34(Suppl.), Abstract 11006 (2016).
  Google Scholar
• 73 Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ. Angiosarcoma. Lancet Oncol. 11(10), 983–991 (2010).
  MedlineGoogle Scholar
• 74 Stacchiotti S, Tamborini E, Marrari A et al. Response to sunitinib malate in advanced alveolar soft part sarcoma. Clin. Cancer Res. 15(3), 1096–1104 (2009).
  Medline CASGoogle Scholar
• 75 Pink D, Bertz-Lepel J, Busemann C, Bitz U, Reichardt P. Efficacy of trabectedin in patients with advanced or metastatic alveolar soft-part sarcoma. Onkologie 35(5), 249–252 (2012).
  Medline CASGoogle Scholar
• 76 Jagodzińska-Mucha P, Świtaj T, Kozak K et al. Long-term results of therapy with sunitinib in metastatic alveolar soft part sarcoma. Tumori 2017 Mar 8:0. doi: 10.5301/tj.5000617 (Epub ahead of print).
  MedlineGoogle Scholar
• 77 Clinical Trial Database: NCT01391962. https://clinicaltrials.gov/ct2/show/NCT01391962.
  Google Scholar
• 78 Clinical Trial Database: NCT03141684. https://clinicaltrials.gov/ct2/show/NCT03141684.
  Google Scholar
• 79 Clinical Trial Database: NCT03016819. https://clinicaltrials.gov/ct2/show/NCT03016819.
  Google Scholar
• 80 Hyman DM, Puzanov I, Subbiah V et al. Vemurafenib in multiple nonmelanoma cancers with BRAF V600 mutations. N. Engl. J. Med. 373(8), 726–736 (2015).
  Medline CASGoogle Scholar
• 81 Robert C, Karaszewska B, Schachter J et al. Improved overall survival in melanoma with combined dabrafenib and trametinib. N. Engl. J. Med. 372(1), 30–39 (2015).
  Medline CASGoogle Scholar