Preclinical studies of DFSP showed that the constitutive activation of the PDGFRB tyrosine kinase domain resulting from t(17;22)(q22;q13) translocation together with COL1A1-PDGFB gene rearrangement was essential for DFSP pathogenesis [11], suggesting that DFSP could be targeted by imatinib. This evidence concerns the gene COL1A1 and dermatofibrosarcoma protuberans.