Targeted treatments of sarcomas and connective tumors beside gastrointestinal stromal tumor.

PURPOSE OF REVIEW: Sarcoma is a heterogeneous group of malignancies historically treated with classic cytotoxic chemotherapy. This review updates the recent advances in targeted therapies in soft-tissue sarcoma, bone sarcoma and other connective diseases with local aggressiveness.

RECENT FINDINGS: Platelet-derived growth factor receptor (PDGFR) inhibitors, antiangiogenics, cell cycle inhibitors and immunomodulatory agents are the main targeted therapies in development in sarcoma. PDGFRα inhibitor olaratumab is being evaluated in a phase III trial in combination with doxorubicin against doxorubicin in monotherapy and, in case of positive results, it could change the standard in the first-line setting. Immunotherapy is still in the early phases of development, although some data in synovial sarcoma are promising. Targeted agents are also in development in other mesenchymal neoplasms, such as the inhibitor of colony stimulating factor 1 receptor for pigmented villonodular synovitis.

SUMMARY: Several targeted therapies are in development in sarcoma and could be added to the therapeutic armamentarium in the near future. However, predictive factors still need to be identified to better select the target population of these new drugs.