Abstract
IntroductionSoft 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.Case presentationA 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/m2/24-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.ConclusionsThis case report illustrates the potential therapeutic activity of trabectedin in patients with myxoid liposarcoma.
Highlights
Soft tissue sarcomas are heterogeneous tumors that are difficult to treat
Trabectedin, which binds to the deoxyribonucleic acid (DNA) minor groove, has demonstrated significant activity in phase II studies in patients with Soft tissue sarcomas (STS), those with leiomyosarcomas and liposarcomas (LPS), leading to its approval for this indication in the European Union [2]
Myxoid liposarcoma (ML) accounts for one-third of liposarcomas and represents a morphologic continuum encompassing myxoid and myxoid/round cell variants, the latter characterized by an extent of round cell components
Summary
Soft tissue sarcomas (STS) are heterogeneous tumors that are difficult to treat. Up to 50 percent of patients develop metastatic disease and require systemic chemotherapy. That required extension surgery up to the periosteum, without the presence of residual tumor in the margins She received adjuvant treatment with external radiotherapy to a dose of 70Gy. Two years later, while pregnant, she noted a mass in her left cervical and right axillary region, which proved to be a metastatic low-grade LPS (Figure 1). A magnetic resonance imaging (MRI) scan showed a 50 percent reduction in the left supraclavicular mass, improvement in the chest lesion and resolution of the pleural effusion This was considered stable disease by RECIST (Response Evaluation Criteria In Solid Tumors) criteria and a PR according to the criteria of Choi et al [5]. Our patient remained asymptomatic with good treatment tolerability (only two episodes of grade 2 and 3 neutropenia in cycles 3 and 4, respectively)
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