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Review
. 2023 Apr;24(4):274-291.
doi: 10.1007/s11864-023-01057-4. Epub 2023 Feb 28.

Myxoid Liposarcomas: Systemic Treatment Options

Elise F Nassif  1   2   3 , Emily Z Keung  3 , Prapassorn Thirasastr  1 , Neeta Somaiah  4
Affiliations
Review

Myxoid Liposarcomas: Systemic Treatment Options

Elise F Nassif et al. Curr Treat Options Oncol. 2023 Apr.

Abstract

Myxoid/round-cell liposarcoma (MRCL) account for 30% of liposarcomas and are the most chemo-sensitive subtype of liposarcoma. The 5-year local relapse and distant metastasis rates are 10% and 20%, respectively. In the advanced setting, the first-line median progression-free survival and overall survival is 9 and 30 months, respectively. The overall response rate (ORR) by RECIST with anthracycline-based chemotherapy is around 40% and with trabectedin is 20%, although response is higher when captured by CHOI criteria. Anthracycline-based combination chemotherapy regimens remain the standard of care first-line treatment option. However, trabectedin is also effective and may be considered in the first-line setting when anthracyclines cannot be prescribed. Beyond chemotherapy, new therapeutic classes are being developed, including autologous adoptive modified T cell receptor cellular therapies which have shown promising results thus far. These new therapies utilize the immunogenic potential of cancer testis antigens, NY-ESO-1 and MAGE-A4, which are expressed in the vast majority of MRCL. Early phase trials have shown encouraging results with up to 40% ORR and a median progression-free survival up to 8.7 months. Other innovative strategies are being developed, tailored to the molecular biology of MRCL. This review summarizes current evidence for the use of standard chemotherapy and the new biomarker-selected treatments under development.

Keywords: Adoptive T cell therapies; Cancer testis antigens; Doxorubicin; Myxoid liposarcoma; Round-cell liposarcoma; Trabectedin.

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    1. Najbauer J, Ducimetière F, Lurkin A, Ranchère-Vince D, Decouvelaere A.-V, Péoc’h M, Istier L, Chalabreysse P, Muller C, Alberti L, 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. https://doi.org/10.1371/journal.pone.0020294 .
    1. Lee ATJ, Thway K, Huang PH, Jones RL. Clinical and molecular spectrum of liposarcoma. J Clin Oncol. 2018;36:151–9. https://doi.org/10.1200/jco.2017.74.9598 . - DOI - PubMed
    1. Pollack SM, Somaiah N, Araujo DM, Druta M, Van Tine BA, Burgess MA, Chawla SP, Seetharam M, Okuno SH, Bohac C, et al. Clinical outcomes of patients with advanced synovial sarcoma or myxoid/round cell liposarcoma treated at major cancer centers in the United States. Cancer Med. 2020;9:4593–602. https://doi.org/10.1002/cam4.3039 . Retrospective multicenter study across nine cancer center in the USA demonstrating heterogeneity of first-line systemic treatment prescribed to patients with MRCL.
    1. WHO Classification of Tumours Editorial Board. Soft Tissue and Bone Tumours, International Agency for Research on Cancer: 2020.
    1. Smith TA, Easley KA, Goldblum JR. Myxoid/round cell liposarcoma of the extremities. Am J Surg Pathol. 1996;20:171–80. https://doi.org/10.1097/00000478-199602000-00005 . - DOI - PubMed

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