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Review
. 2020 Feb;27(Suppl 1):25-33.
doi: 10.3747/co.27.5475. Epub 2020 Feb 1.

Update on systemic therapy for advanced soft-tissue sarcoma

A Smrke  1 , Y Wang  1 , C Simmons  1
Affiliations
Review

Update on systemic therapy for advanced soft-tissue sarcoma

A Smrke et al. Curr Oncol. 2020 Feb.

Abstract

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.

Keywords: Soft-tissue sarcoma; advanced; anthracyclines; systemic therapy.

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Conflict of interest statement

CONFLICT OF INTEREST DISCLOSURES We have read and understood Current Oncology’s policy on disclosing conflicts of interest, and we declare that we have none.

Figures

FIGURE 1
FIGURE 1
Consort diagram for the scoping review. STS = soft-tissue sarcoma; ES = Ewing sarcoma; RMS = rhabdomyosarcoma; GIST = gastrointestinal stromal tumor; DF = desmoid fibromatosis.
FIGURE 2
FIGURE 2
(A) Types of published study included (n = 62). (B) Trends in publication per year since 1987. (C) Class of systemic therapy used in the study. TKI = tyrosine kinase inhibitor.
FIGURE 3
FIGURE 3
Proposed treatment algorithm for advanced soft-tissue sarcoma (STS). aDacarbazine, gemcitabine, pazopanib, trabectedin. NGS = next-generation sequencing; PS = performance status; LMS = leiomyosarcoma; LPS = liposarcoma; MDC = multidisciplinary conference.
See this image and copyright information in PMC

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