Myxoid Liposarcomas: Systemic Treatment Options
Elise F Nassif1,2,3, Emily Z Keung3, Prapassorn Thirasastr1
1Department of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, 1400 Holcombe Blvd, Houston, TX, USA.
Opinion Statement:
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.
Insights
Myxoid/round-cell liposarcoma (MRCL) is the most chemo-sensitive subtype. Current treatments include anthracycline chemotherapy and trabectedin, with new T cell therapies showing promise.
Area of Science:
- Oncology
- Medical Research
Background:
- Myxoid/round-cell liposarcoma (MRCL) constitutes 30% of liposarcomas and is the most chemo-sensitive subtype.
- MRCL presents a 5-year local relapse rate of 10% and distant metastasis rate of 20%.
Purpose of the Study:
- To review current evidence on standard chemotherapy for MRCL.
- To summarize emerging biomarker-selected treatments for MRCL.
Main Methods:
- Review of current evidence on standard chemotherapy regimens.
- Summary of novel therapeutic classes, including T cell receptor cellular therapies.
Main Results:
- Anthracycline-based chemotherapy offers an overall response rate (ORR) of ~40% by RECIST criteria.
- Trabectedin shows a 20% ORR by RECIST, with higher response rates by CHOI criteria.
- Emerging T cell therapies targeting NY-ESO-1 and MAGE-A4 show up to 40% ORR and 8.7 months median progression-free survival.
Conclusions:
- Anthracycline-based combination chemotherapy remains the first-line standard for MRCL.
- Trabectedin is an alternative first-line option when anthracyclines are contraindicated.
- Novel therapies, particularly T cell receptor cellular therapies, demonstrate promising results and warrant further investigation.
