Trabectedin in myxoid liposarcomas (MLS): a long-term analysis of a single-institution series

Abstract

These data indicate that the high response rate of MLS to trabectedin translates into prolonged PFS. Surgery of residual metastatic disease is already used quite extensively in metastatic MLS. Trabectedin may give further significance to this kind of surgery.

A Review of Trabectedin (ET-743): A Unique Mechanism of Action

“…The overall response rate to trabectedin observed in several phase II studies in patients who have failed one or two lines of chemotherapy is around 10%, with disease stabilization exceeding 50%. The more relevant antitumor efficacy is observed in patients with advanced myxoid liposarcoma, in which trabectedin induces objective responses in approximately 50% of cases and progression-free survival exceeding 2 years in 80% of patients (25,26). Myxoid liposarcoma pathogenesis is related to characteristic chromosomal translocations such as t(12;16)(q13;p11) or less frequently t(12;22)(q13;q12), resulting in the expression of FUS-CHOP and EWS-CHOP fusion genes, respectively (27).…”

Trabectedin in patients with pre-treated advanced soft tissue sarcoma: a retrospective single center analysis

“…Generally, trabectedin has shown slightly better efficacy in leiomyosarcoma and liposarcoma subtypes compared to other STS subtypes [12,21]. Trabectedin has shown notable efficacy in myxoid liposarcomas [11,12].…”

“…That may mean that a group of patients can benefit from long lasting maintenance therapy, which can be challenging taking into account the pharmacoeconomics of the drug. The clinician is thus left with the open issue of how long therapy should be continued in responding/stable patients [12], especially as the toxicity of the drug, in contrast to many traditional chemotherapeutics, is not cumulative [15,21].…”

“…Although the objective response rate in patients with anthracycline-resistant advanced STS has not exceeded 10% in multiple phase I and II clinical trials, this drug has resulted in control of disease with progression arrest rates exceeding 50% and progression-free survival (PFS) exceeding 20% at 6 months [10]. The main benefits have been documented in leiomyosarcoma and liposarcoma (which are the most common adult sarcoma subtypes); particular sensitivity to the drug was noted in myxoid liposarcoma subtype [11,12].…”

“…All patients were previously treated with chemotherapy. R Re es su ul lt ts s: : 120 courses of trabectedin were given (median 4, range [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]. We were able to assess the response in 24 patients.…”