Thérapies ciblées et dermatofibrosarcome de Darier et Ferrand

摘要

Dermatofibrosarcoma is a rare low-grade sarcoma characterized in more than 90% of cases by (17q22; 22q13) translocation leading to a fusion transcript COL1A1-PDGFB. Surgical therapy is the standard of care; however, tyrosine-kinase inhibitors targeting PDGFR are of interest in unresectable or rare metastatic tumors. Imatinib mesylate has been approved in this indication and research is going on with other inhibitors.