Update on Systemic Therapy for Advanced Soft Tissue Sarcoma

Background : Soft tissue sarcoma (STS) is a rare group of mesenchymal neoplasms which contains over 50 heterogenous subtypes.  There have been great efforts to increase the understanding of treatment of advanced STS (unresectable or metastatic disease). We wished to determine if outcomes for patients with advanced STS have improved over time, and to assess the current evidence for systemic therapy.Methods : We performed a scoping review to evaluate the contemporary evidence for systemic treatment of advanced soft tissue in adults (u003e18 years old). 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 77 studies, 62 trials met inclusion criteria. Results : The number of clinical trials conducted and published in advanced STS has increased over the last 30 years. Although median OS 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 inherent heterogeneity of grouping multiple STS subtypes within clinical trials. However, increasing numbers of agents are being studied and several studies had shown isolated PFS or OS benefit. Conclusions : First line anthracycline systemic therapy remains the standard of care for advanced STS.  However, choice of subsequent therapy beyond anthracyclines remains challenging. Novel systemic therapies, using molecular diagnostics to direct therapy, subtype specific trials and learning from real world retrospective data are all important in improving outcomes for patients with advanced STS.