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.