Dermatofibrosarcoma protuberans: A surgical disease with a molecular savior

Abstract

PURPOSE OF REVIEW: Dermatofibrosarcoma protuberans is a low-grade malignancy of the skin and subcutaneous tissues with low potential for the development of distant metastases. This tumor is characterized by infiltrative growth, and a chromosomal translocation involving the COL1A1 gene on chromosome 17 and the gene platelet-derived growth factor B gene on chromosome 22. This review will examine recent data confirming the central role of surgery in managing this disease and new findings for the application of molecularly targeted therapy in patients with unresectable disease. RECENT FINDINGS: The largest published series of dermatofibrosarcoma protuberans cases has reported that surgery can le..