TY - JOUR A2 - Kovar, H. AU - Rutkowski, Piotr AU - Wozniak, Agnieszka AU - Switaj, Tomasz PY - 2011 DA - 2011/03/30 TI - Advances in Molecular Characterization and Targeted Therapy in Dermatofibrosarcoma Protuberans SP - 959132 VL - 2011 AB - The molecular pathogenesis of dermatofibrosarcoma protuberans (DFSP) involves distinctive rearrangement of chromosomes 17 and 22 leading to formation of the COL1A1-PDGFB fusion gene. The knowledge of molecular events underlying development of DFSP resulted in the implementation of targeted therapy with imatinib—a tyrosine kinase inhibitor (TKI), to the clinical practice. The striking efficacy of imatinib in advanced cases of DFSP has been demonstrated in a few clinical trials. Thus, imatinib is currently considered the gold standard in the treatment of inoperable and/or metastatic and/or recurrent cases of DFSP. Therapy with imatinib may potentially facilitate resection or decrease possible disfigurement related to radical surgical procedure. Following partial response on imatinib significant percentage of patients may be rendered free of the disease by surgery of the residual tumor. SN - 1357-714X UR - https://doi.org/10.1155/2011/959132 DO - 10.1155/2011/959132 JF - Sarcoma PB - Hindawi Publishing Corporation KW - ER -