Current Oncology Reports

, Volume 8, Issue 4, pp 291–296 | Cite as

Targeted therapy for dermatofibrosarcoma protuberans

  • Thomas A. Abrams
  • Scott M. Schuetze


Dermatofibrosarcoma protuberans (DFSP) is a rare, cutaneous tumor characterized by aggressive local invasion. Local recurrence after excision is common, especially when negative margins are not achieved. DFSP frequently exhibits translocation of chromosomes 17 and 22, t(17;22). This rearrangement fuses the collagen type Ià1 (COL1A1) gene to the platelet-derived growth factor B-chain (PDGFB) gene. The resultant chimeric gene causes unregulated expression of platelet-derived growth factor leading to abnormal activation of the platelet-derived growth factor receptor (PDGFR) â tyrosine kinase through an autocrine loop. This is believed to be the critical event in DFSP tumorigenesis. Imatinib mesylate is a potent inhibitor of several protein tyrosine kinases, including the PDGFRs. Clinical evidence suggests that imatinib mesylate is a safe and effective treatment in DFSP, especially in cases of recurrent or metastatic disease. Three phase II, multicenter clinical trials are open to further investigate the role of imatinib mesylate in DFSP.


Imatinib Protein Tyrosine Kinase Imatinib Mesylate Negative Margin Wide Local Excision 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Current Science Inc 2006

Authors and Affiliations

  1. 1.Division of Hematology/Oncology, Department of MedicineUniversity of Michigan Comprehensive Cancer CenterAnn ArborUSA

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