Targeted therapy for dermatofibrosarcoma protuberans

被引:17
作者
Abrams T.A. [1 ]
Schuetze S.M. [1 ]
机构
[1] Division of Hematology and Oncology, Department of Medicine, University of Michigan Comprehensive Cancer Center, Ann Arbor, MI 48109-0848
关键词
Imatinib; Protein Tyrosine Kinase; Imatinib Mesylate; Negative Margin; Wide Local Excision;
D O I
10.1007/s11912-006-0035-3
中图分类号
学科分类号
摘要
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 1α1 (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) β 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. Copyright © 2006 by Current Science Inc.
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页码:291 / 296
页数:5
相关论文
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