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Abstract
Soft tissue sarcomas are rare cancers of mesenchymal origin. Recent progress in the understanding of the biology of these rare tumours has enabled the identification of distinct molecular and pathological entities within this heterogenous group of neoplasms, and has paved the way for the development of targeted therapeutics directed against activated kinases. One of the most clear examples is the identification of KIT and platelet-derived growth factor receptor-α kinase mutations in gastrointestinal stromal tumours, a subset of sarcomas arising from precursors of the interstitial cells of Cajal in the digestive tract, which led to the development of imatinib, sunitinib and other tyrosine kinase inhibitors for the treatment of solid tumours. This model has become the paradigm of a targeted treatment of solid tumours designed to inhibit the causal alteration in the oncogenesis of these tumours. This review summarises treatment strategies in the context of advanced disease and discusses new compounds being developed for patients with soft tissue sarcomas.