| Summary: | <p>Abstract</p> <p>Introduction</p> <p>Soft tissue sarcomas are heterogeneous tumors that are difficult to treat. Up to 50 percent of patients develop metastatic disease and require systemic chemotherapy. Ifosfamide and doxorubicin are the two most active agents.</p> <p>Case presentation</p> <p>A 33-year-old Caucasian woman presented to our facility with a metastatic myxoid liposarcoma. Our patient was initially treated with surgery and radiation therapy, but experienced three recurrences during a six-year period, the first and the last occurring while our patient was pregnant. The first recurrence, which occurred two years after diagnosis and was localized in the left cervical and right axillary region, was treated with surgery followed by chemotherapy. Molecular analysis of this tumor showed a t(12,16) + translocation resulting in a FUS-DDIT3 or EWSR1-DDIT3 fusion. Three years later our patient experienced a second recurrence in the left supraclavicular fossa, upper thoracic and anterior mediastinum, which was treated with surgery alone. Eight months later, during the second pregnancy, our patient experienced a third recurrence as a large cervical mass that was treated, upon pregnancy, with trabectedin (1.5mg/m<sup>2</sup>/24-hour continuous infusion) for a total of 12 cycles. At that time a computed tomography scan showed long-term partial response with excellent treatment tolerability.</p> <p>Conclusions</p> <p>This case report illustrates the potential therapeutic activity of trabectedin in patients with myxoid liposarcoma.</p>
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