Abstract
Introduction: Adult sarcomas are rare tumors characterized, in general, by their poor prognosis and the paucity of effective treatments. However, the deeper understanding of their underlying molecular pathology, signaling pathways and key effectors has permitted the development of a number of drugs able to inhibit important processes in sarcoma pathogenesis. Some of these novel compounds have been assessed in clinical trials with successful results.
Areas covered: The latest reported trials are comprehensively reviewed. Thus, the Phase III studies with pazopanib, regorafenib, muramyl tripeptide (MTP) and ridaforolimus are extensively discussed as well as the biological rationale for the use of these compounds. In addition, the most promising drugs that still are in earlier stages of development such as CDK4 and MDM2 inhibitors, cediranib, eribulin and crizotinib are also discussed.
Expert opinion: It is crucial for the correct identification of active drugs in sarcomas that new clinical trials are focused on specific subtypes and/or molecular alterations. The results of these studies should improve the prognosis of the patients affected by sarcoma in forthcoming years.
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