Soft tissue sarcomas (STS) are rare malignant tumors arising from mesenchymal tissues and have an overall incidence of about 1.15/100 000 per year. According to the World Health Organization classification

over 100 distinct histological subtypes have been categorized

many molecular aberrations are prevalent within specific sarcoma

and very few are therapeutically targeted. Instead of utilizing molecular markers as targets

first-line sarcoma treatment options are still limited to traditional surgery and chemotherapy. Current understanding of the underlying molecular and genomic mechanisms of different histology subtypes have led to encouraging development of targeted therapy for STS. In this review

we explained and summarized the current molecular targeted therapies in development for the most common studied subtypes of soft tissue sarcoma.