Background: Soft-tissue sarcoma (
sts) represents a rare group of mesenchymal neoplasms comprising more than 50 heterogeneous subtypes. Great efforts have been made to increase the understanding of the treatment of advanced
sts (unresectable or metastatic disease). We set out to determine whether outcomes for patients with advanced
sts have improved over time and to assess the current evidence for systemic therapy.
Methods: In a scoping review, we evaluated the contemporary evidence for systemic treatment of advanced
sts in adults (>18 years of age). Phase
i,
ii, and
iii studies of systemic therapy for advanced
sts published in the English language were included. After abstract and full-text review of seventy-seven studies, sixty-two trials met the inclusion criteria.
Results: The number of clinical trials conducted and published in advanced
sts has increased over the last 30 years. Although median overall survival has increased, attempts at improving first-line therapy through dose intensification, doublet chemotherapy, or alternative backbones have not been successful. The optimal therapy beyond anthracyclines remains a challenge, especially given the heterogeneity that grouping multiple
sts subtypes within clinical trials creates. However, increasing numbers of agents are being studied, and several studies had shown isolated benefit in progression-free or overall survival.
Summary: First-line systemic therapy with an anthracycline remains the standard of care for advanced
sts. However, choice of subsequent therapy beyond anthracyclines remains challenging. Novel systemic therapies, use of molecular diagnostics to direct therapy, subtype-specific trials, and learnings from real-world retrospective data are all important for improving outcomes in patients with advanced
sts.
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