The Role of Systemic Therapies in the Management of Soft Tissue Sarcoma

Abstract

Soft tissue sarcomas (STS) are solid tumours that originate from mesenchymal cells and can be subclassified into over 50 subtypes. Representing 1% of adult tumours and 15% of paediatric malignancies, their rarity and diversity has traditionally made clinical trial design and thereby the provision of a strong evidence base to guide individual patient management challenging. As therapeutic options as a whole have increased for solid cancers in the context of an increased understanding of molecular drivers that predict response to those therapies, the challenges faced by the STS community have meant that the overall pace of drug development has been slower, with some notable exceptions in selected sarcoma subtypes in which clear molecular drivers have been identified (e.g. gastrointestinal stromal tumours). However, the recognition that “splitting” these requisite subtypes even if based on classical histopathological features into their respective individual entities over the last two decades, rather than “lumping” all STS together, has helped advance the field considerably. In addition, international collaborative efforts have advanced in parallel, facilitating the successful conduct of trials in specific subtypes to be conducted, including randomized phase III trials.