Journal article

Dermatofibrosarcoma protuberans: Recent clinical progress

Grant McArthur

ANNALS OF SURGICAL ONCOLOGY | SPRINGER | Published : 2007

Abstract

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor of low malignant grade characterized by a pattern of slow, infiltrative growth and a marked tendency to recur locally after surgical excision. Wide surgical resection is generally accepted as optimal treatment for DFSP. However, despite optimal surgical management, distant metastases may develop in up to 5% of patients. More than 90% of DFSP are characterized by a reciprocal chromosomal translocation, t(17;22). This rearrangement leads to constitutive activation of the platelet-derived growth factor receptor (PDGFR) as a result of deregulated ligand expression, thus providing a rationale for targeted inhibition of P..

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University of Melbourne Researchers