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memo - Magazine of European Medical Oncology

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15.04.2020 | short review

Metastatic sarcoma: tailored strategies for a heterogeneous disease

verfasst von: Massimiliano Grassi, Andrea Spagnoletti, Alberto Puccini, MD

Erschienen in: memo - Magazine of European Medical Oncology | Ausgabe 2/2020

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Summary

Soft tissue sarcomas are rare diseases that encompass a very heterogeneous group of tumors with diverse pathology and clinically overlapping characteristics. Although some treatment options may be used across different subtypes, each is characterized by specific features and may benefit more from specific approaches. In such a rare and peculiar group of diseases, it is rather important to define the best strategy to prolong survival and improve the quality of life of the patients. Although anthracycline-based chemotherapy remains a milestone in the first-line setting, in recent years novel targeted and immune therapies have been developed and more tailored strategies are possible. For these reasons, every case should be discussed in a multidisciplinary board that includes all specialists involved in the treatment of these patients, since the combination of systemic and local treatments can often be proposed. In this short review, we present the state of the art and offer future perspectives for the management of soft tissue sarcomas.

Vorheriger Artikel Perioperative treatment of soft-tissue sarcoma

Nächster Artikel Therapy management in bone sarcoma

Fletcher CD, Hogendoorn PC, Mertens F, Bridge J. WHO classification of tumours of soft tissue and bone. 4th ed. Lyon: IARC press; 2013.

Burningham Z, Hashibe M, Spector L, et al. The epidemiology of sarcoma. Clin Sarcoma Res. 2012;2:14. https://doi.org/10.1186/2045-3329-2-14.CrossRefPubMedPubMedCentral

Chao C, Al-Saleem T, Brooks JJ, et al. Vascular endothelial growth factor and soft tissue sarcomas: tumor expression correlates with grade. Ann Surg Oncol. 2001;8(3):260–7. https://doi.org/10.1007/s10434-001-0260-9.CrossRefPubMed

Pakos EE, Goussia AC, Tsekeris PG, Papachristou DJ, Stefanou D, Agnantis NJ. Expression of vascular endothelial growth factor and its receptor, KDR/Flk‑1, in soft tissue sarcomas. Anticancer Res. 2005;25(5):3591–6.PubMed

Quesada J, Amato R. The molecular biology of soft-tissue sarcomas and current trends in therapy. Sarcoma. 2012; https://doi.org/10.1155/2012/849456.CrossRefPubMedPubMedCentral

Frezza AM, Stacchiotti S, Gronchi A. Systemic treatment in advanced soft tissue sarcoma: what is standard, what is new. BMC Med. 2017;15(1):109. https://doi.org/10.1186/s12916-017-0872-y.CrossRefPubMedPubMedCentral

Benjamin RS, Wiernik PH, Bachur NR. Adriamycin: a new effective agent in the therapy of disseminated sarcomas. Med Pediatr Oncol. 1975;1(1):63–76.CrossRef

Borden EC, Amato DA, Rosenbaum C, et al. Randomized comparison of three adriamycin regimens for metastatic soft tissue sarcomas. J Clin Oncol. 1987;5(6):840–50.CrossRef

Judson I, Verweij J, Gelderblom H, et al. 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. 2014;15:415–23.CrossRef

10.

Lorigan P, Verweij J, Papai Z, et al. 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. 2007;25:3144–50.CrossRef

11.

Wilding CP, Elms ML, Judson I, et al. The landscape of tyrosine kinase inhibitors in sarcomas: looking beyond pazopanib. Expert Rev Anticancer Ther. 2019;19(11):971–91. https://doi.org/10.1080/14737140.2019.1686979.CrossRefPubMedPubMedCentral

12.

West RB, Rubin BP, Miller MA, et al. A landscape effect in tenosynovial giant-cell tumor from activation of CSF1 expression by a translocation in a minority of tumor cells. Proc Natl Acad Sci U S A. 2006;103(3):690–5.CrossRef

13.

Cupp JS, Miller MA, Montgomery KD, et al. Translocation and expression of CSF1 in pigmented villonodular synovitis, tenosynovial giant cell tumor, rheumatoid arthritis and other reactive synovitides. Am J Surg Pathol. 2007;31(6):970–6.CrossRef

14.

Verspoor FGM, Mastboom MJL, Hannink G, et al. Long-term efficacy of imatinib mesylate in patients with advanced tenosynovial giant cell tumor. Sci Rep. 2019;9(1):14551. https://doi.org/10.1038/s41598-019-51211-y.CrossRefPubMedPubMedCentral

15.

Tap WD, Gelderblom H, Palmerini E, et al. Pexidartinib versus placebo for advanced tenosynovial giant cell tumour (ENLIVEN): a randomised phase 3 trial. Lancet. 2019;394(10197):478–87.CrossRef

16.

Lamb YN. Pexidartinib: first approval. Drugs. 2019;79:1805–12. https://doi.org/10.1007/s40265-019-01210-0.CrossRefPubMedPubMedCentral

17.

Navarrete-Dechent C, Mori S, Barker CA, Dickson MA, Nehal KS. Imatinib treatment for locally advanced or metastatic dermatofibrosarcoma protuberans: a systematic review. JAMA Dermatol. 2019;155(3):361–9. https://doi.org/10.1001/jamadermatol.2018.4940.CrossRefPubMed

18.

Stacchiotti S, Tortoreto M, Baldi GG, et al. Preclinical and clinical evidence of activity of pazopanib in soft fibrous tumour. Eur J Cancer. 2014;50:3021–8.CrossRef

19.

Stacchiotti S, Negri T, Libertini M, et al. Sunitinib malate in solitary fibrous tumor (SFT). Ann Oncol. 2012;23:3171–9.CrossRef

20.

Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M. Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor. Oncol Rev. 2014;8(2):259. https://doi.org/10.4081/oncol.2014.259.CrossRefPubMedPubMedCentral

21.

Cousin S, Le Loarer F, Crombé A, et al. Epithelioid hemangioendothelioma. Bull Cancer. 2019;106(1):73–83. https://doi.org/10.1016/j.bulcan.2018.11.004.CrossRefPubMed

22.

Rosenberg A, Agulnik M. Epithelioid hemangioendothelioma: update on diagnosis and treatment. Curr Treat Options Oncol. 2018;19:19. https://doi.org/10.1007/s11864-018-0536-y.CrossRefPubMed

23.

Kollár A, Jones RL, Stacchiotti S, et al. Pazopanib in advanced vascular sarcomas: an EORTC Soft Tissue and Bone Sarcoma Group (STBSG) retrospective analysis. Acta Oncol 2017; 56(1):88–92. https://doi.org/10.1080/0284186X.2016.1234068.

24.

Chevreau C, Le Cesne A, Ray-Coquard I, et al. Sorafenib in patients with progressive epithelioid hemangioendothelioma: a phase 2 study by the French Sarcoma Group. Cancer. 2013;119:2639–44.CrossRef

25.

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;24:257–63.CrossRef

26.

Radzikowska E, Szczepulska-Wójcik E, Chabowski M, et al. Pulmonary epithelioid haemangioendothelioma—interferon 2‑alpha treatment—case report. Pneumonol Alergol Pol. 2008;76(4):281–5.PubMed

27.

Stacchiotti S, Provenzano S, Dagrada G, et al. Sirolimus in advanced epithelioid hemangioendothelioma: a retrospective case-series analysis from the Italian rare cancer network database. Ann Surg Oncol. 2016;23:2735–44. https://doi.org/10.1245/s10434-016-5331-z.CrossRefPubMed

28.

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. https://doi.org/10.1200/JCO.2009.25.2981.CrossRefPubMedPubMedCentral

29.

Benson C, Vitfell-Rasmussen J, Maruzzo M, et al. A retrospective study of patients with malignant PEComa receiving treatment with sirolimus or temsirolimus: the Royal Marsden Hospital experience. Anticancer Res. 2014;34(7):3663–8.PubMed

30.

Singhi EK, Moore DC, Muslimani A. Metastatic soft tissue sarcomas: a review of treatment and new pharmacotherapies. P T. 2018;43(7):410–29.PubMedPubMedCentral

31.

Meyer M, Seetharam M. First-line therapy for metastatic soft tissue sarcoma. Curr Treat Options Oncol. 2019;20:6. https://doi.org/10.1007/s11864-019-0606-9.CrossRefPubMed

32.

Lee SH, Chang MH, Baek KK, et al. High-dose ifosfamide as second- or third-line chemotherapy in refractory bone and STS patients. Oncology. 2011;80:257–61.CrossRef

33.

van Oosterom AT, Mouridsen HT, Nielsen OS, et al. Results of randomised studies of the EORTC Soft Tissue and Bone Sarcoma Group (STBSG) with two different ifosfamide regimens in first-and second-line chemotherapy in advanced STS patients. Eur J Cancer. 2002;38:2397–406.CrossRef

34.

Rosen G, Forscher C, Lowenbraun S, et al. Synovial sarcoma. Uniform response of metastases to high dose ifosfamide. Cancer. 1994;73:2506–11. https://doi.org/10.1002/1097-0142(19940515)73:10.CrossRefPubMed

35.

Duffaud F, Pautier P, Bui B, et al. A pooled analysis of the final results of the two randomized phase II studies comparing Gemcitabine (G) vs Gemcitabine + Docetaxel (G + D) in patients (pts) with metastatic/relapsed leiomyosarcoma (LMS). Milan: European Society for Medical Oncology; 2010.

36.

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;23:501–8. https://doi.org/10.1093/annonc/mdr066.CrossRefPubMed

37.

Bui-Nguyen B, Butrynski JE, Penel N, et al. A phase IIb multicentre study comparing the efficacy of trabectedin to doxorubicin in patients with advanced or metastatic untreated soft tissue sarcoma: the TRUSTS trial. Eur J Cancer. 2015;51:1312–20. https://doi.org/10.1016/j.ejca .2015.03.023.CrossRef

38.

Forni C, Minuzzo M, Virdis E, et al. Trabectedin (ET-743) promotes differentiation in myxoid liposarcoma tumors. Mol Cancer Ther. 2009;8:449–57.CrossRef

39.

Di Giandomenico S, Frapolli R, Bello E, et al. Mode of action of trabectedin in myxoid liposarcomas. Oncogene. 2014;33:5201–10. https://doi.org/10.1038/onc.2013.462.CrossRefPubMed

40.

Schoffski P, Chawla S, Maki RG, et al. Eribulin versus dacarbazine in previously treated patients with advanced liposarcoma or leiomyosarcoma: a randomised, open-label, multicentre, phase 3 trial. Lancet. 2016;387:1629–37. https://doi.org/10.1016/S0140-6736(15)01283-0.CrossRefPubMed

41.

Dybdal-Hargreaves NF, Risinger AL, Mooberry SL. Eribulin mesylate: mechanism of action of a unique microtubule-targeting agent. Clin Cancer Res. 2015;21:2445–52.CrossRef

42.

Yoshida T, Ozawa Y, Kimura T, et al. Eribulin mesilate suppresses experimental metastasis of breast cancer cells by reversing phenotype from epithelial-mesenchymal transition (EMT) to mesenchymal-epithelial transition (MET) states. Br J Cancer. 2014;110:1497–505.CrossRef

43.

Laughney AM, Kim E, Sprachman MM, et al. Single-cell pharmacokinetic imaging reveals a therapeutic strategy to overcome drug resistance to the microtubule inhibitor eribulin. Sci Transl Med. 2014;6(261):261ra152. https://doi.org/10.1126/scitranslmed.3009318.CrossRefPubMedPubMedCentral

44.

D’Angelo SP, Munhoz RR, Kuk D, et al. Outcomes of systemic therapy for patients with metastatic angiosarcoma. Oncology. 2015;89(4):205–14. https://doi.org/10.1159/000381917.CrossRefPubMedPubMedCentral

45.

Dickson MA, D’Adamo DR, Keohan ML, et al. Phase II trial of gemcitabine and docetaxel with bevacizumab in soft tissue sarcoma. Sarcoma. 2015;2015:532478. https://doi.org/10.1155/2015/532478.CrossRefPubMedPubMedCentral

46.

Cao J, Wang J, He C, Fang M. Angiosarcoma: a review of diagnosis and current treatment. Am J Cancer Res. 2019;9(11):2303–13.PubMedPubMedCentral

47.

Apice G, Pizzolorusso A, Di Maio M, et al. Confirmed activity and tolerability of weekly paclitaxel in the treatment of advanced angiosarcoma. Sarcoma. 2016;2016:6862090. https://doi.org/10.1155/2016/6862090.CrossRefPubMedPubMedCentral

48.

Penel N, Italiano A, Ray-coquard I, et al. Metastatic angiosarcomas: doxorubicin-based regimens, weekly paclitaxel and metastasectomy significantly improve the outcome. Ann Oncol. 2012;23(2):517–23. https://doi.org/10.1093/annonc/mdr138.CrossRefPubMed

49.

Fata F, O’Reilly E, Ilson D, et al. Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face. Cancer. 1999;86(10):2034–7. https://doi.org/10.1002/(sici)1097-0142(19991115)86:10.CrossRefPubMed

50.

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. https://doi.org/10.1016/j.ejca.2008.07.037.CrossRefPubMed

51.

Penel N, Bui BN, Bay J‑O, et al. Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX study. J Clin Oncol. 2008;26(32):5269–74. https://doi.org/10.1200/jco.2008.17.3146.CrossRefPubMed

52.

Italiano A, Cioffi A, Penel N, et al. Comparison of doxorubicin and weekly paclitaxel efficacy in metastatic angiosarcomas. Cancer. 2012;118(13):3330–6. https://doi.org/10.1002/cncr.26599.CrossRefPubMed

53.

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.CrossRef

54.

Doebele RC, Drilon A, Paz-Ares L, et al. Entrectinib in patients with advanced or metastatic NTRK fusion-positive solid tumours: integrated analysis of three phase 1–2 trials. Lancet Oncol. 2020;21(2):271–82. https://doi.org/10.1016/S1470-2045(19)30691-6.CrossRefPubMed

55.

Hong DS, Bauer TM, Lee JJ, et al. Larotrectinib in adult patients with solid tumours: a multi-centre, open-label, phase I dose-escalation study. Ann Oncol. 2019;30(2):325–31. https://doi.org/10.1093/annonc/mdy539.CrossRefPubMedPubMedCentral

56.

Shang H, Braggio D, Lee YJ, et al. Targeting the Notch pathway: a potential therapeutic approach for desmoid tumors. Cancer. 2015;121(22):4088–96. https://doi.org/10.1002/cncr.29564.CrossRefPubMedPubMedCentral

57.

Kummar S, O’Sullivan Coyne G, Do KT, et al. Clinical activity of the γ‑secretase inhibitor PF-03084014 in adults with desmoid tumors (aggressive fibromatosis). J Clin Oncol. 2017;35(14):1561–9. https://doi.org/10.1200/JCO.2016.71.1994.CrossRefPubMedPubMedCentral

58.

Messersmith WA, Shapiro GI, Cleary JM, et al. A phase I, dose-finding study in patients with advanced solid malignancies of the oral γ‑secretase inhibitor PF-03084014. Clin Cancer Res. 2015;21:60–7. https://doi.org/10.1158/1078-0432.CCR-14-0607.CrossRefPubMed

59.

Reichardt P, Lindner T, Pink D, et al. Chemotherapy in alveolar soft part sarcomas. What do we know? Eur J Cancer. 2003;39:1511–6.CrossRef

60.

Agulnik M, Tannir NM, Pressey JG, et al. A phase II, multicenter study of the EZH2 inhibitor tazemetostat in adult subjects with INI1-negative tumors or relapsed/refractory synovial sarcoma. J Clin Oncol. 2016;34(suppl):abstr TPS11071.CrossRef

61.

D’Angelo SP, Mahoney MR, Van Tine BA, et al. Nivolumab with or without ipilimumab treatment for metastatic sarcoma (Alliance A091401): two open-label, non-comparative, randomised, phase 2 trials. Lancet Oncol. 2018;19(3):416–26. https://doi.org/10.1016/S1470-2045(18)30006-8.CrossRefPubMedPubMedCentral

62.

Tawbi HAH, Burgess M, 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.CrossRef

63.

Paoluzzi L, Cacavio A, Ghesani M, et al. Response to anti-PD1 therapy with nivolumab in metastatic sarcomas. Clin Sarcoma Res. 2016;6:24. https://doi.org/10.1186/s13569-016-0064-0.CrossRefPubMedPubMedCentral

64.

Pollack S, Redman MW, Wagner M, et al. A phase I/II study of pembrolizumab (Pem) and doxorubicin (Dox) in treating patients with metastatic/unresectable sarcoma. J Clin Oncol. 2019;37(15_suppl):11009. https://doi.org/10.1200/JCO.2019.37.15_suppl.11009.CrossRef

65.

Gordon EM, Chua-Alcala VS, Kim K, et al. SAINT: results of a phase 1/2 study of safety/efficacy using safe amounts of ipilimumab, nivolumab, and trabectedin as first-line treatment of advanced soft tissue sarcoma. J Clin Oncol. 2019;37(15_suppl):11016. https://doi.org/10.1200/JCO.2019.37.15_suppl.11016.CrossRef

66.

Wilky BA, Trucco MM, Subhawong TK, et al. Axitinib plus pembrolizumab in patients with advanced sarcomas including alveolar soft-part sarcoma: a single-centre, single-arm, phase 2 trial. Lancet Oncol. 2019;20(6):837–48. https://doi.org/10.1016/S1470-2045(19)30153-6.CrossRefPubMed

67.

Kelly CM, Antonescu CR, Bowler T, et al. Objective response rate among patients with locally advanced or metastatic sarcoma treated with talimogene laherparepvec in combination with pembrolizumab: a phase 2 clinical trial. JAMA Oncol. 2020; https://doi.org/10.1001/jamaoncol.2019.6152.CrossRefPubMed

68.

Iura K, Maekawa A, Kohashi K, et al. Cancer-testis antigen expression in synovial sarcoma: NY-ESO‑1, PRAME, MAGEA4 and MAGEA1. Hum Pathol. 2017;61:130–9. https://doi.org/10.1016/j.humpath.2016.12.006.CrossRefPubMed

69.

Chung PWM, Deheshi BM, Ferguson PC, et al. Radiosensitivity translates into excellent local control in extremity myxoid liposarcoma. Cancer 2009; 115:3254–61. https://doi.org/10.1002/cncr.24375.

70.

Dudek W, Schreiner W, Mykoliuk I, Higaze M, Sirbu H. Pulmonary metastasectomy for sarcoma-survival and prognostic analysis. J Thorac Dis. 2019;11(8):3369–76. https://doi.org/10.21037/jtd.2019.08.10.CrossRefPubMedPubMedCentral

71.

Miner TJ, Brennan MF, Jaques DP. A prospective, symptom related, outcomes analysis of 1022 palliative procedures for advanced cancer. Ann Surg. 2004;240:719–26. https://doi.org/10.1097/01.sla.0000141707.09312.dd. discussion 726.CrossRefPubMedPubMedCentral

Titel: Metastatic sarcoma: tailored strategies for a heterogeneous disease
verfasst von: Massimiliano Grassi
Andrea Spagnoletti
Alberto Puccini, MD
Publikationsdatum: 15.04.2020
Verlag: Springer Vienna
Erschienen in: memo - Magazine of European Medical Oncology / Ausgabe 2/2020
Print ISSN: 1865-5041
Elektronische ISSN: 1865-5076
DOI: https://doi.org/10.1007/s12254-020-00598-w

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Current state and perspectives of checkpoint inhibitors in ovarian cancer treatment

PLATON: use of romiplostim to treat chronic primary immune thrombocytopenia

Diagnostic and therapeutic management of sarcoma patients

Hepatocellular carcinoma

Perioperative treatment of soft-tissue sarcoma

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