DERMATOFIBROSARCOMA PROTUBERANS

Course overview

DERMATOFIBROSARCOMA PROTUBERANS (DFSP) IS AN UNCOMMON SOFT TISSUE TUMOR THAT INVOLVES THE DERMIS, SUBCUTANEOUS FAT, AND IN RARE CASES, MUSCLE AND FASCIA. THE TUMOR TYPICALLY PRESENTS AS A SLOWLY GROWING, FIRM PLAQUE ON THE TRUNK OF YOUNG ADULTS. THE CAUSE OF DERMATOFIBROSARCOMA PROTUBERANS IS NOT CLEARLY UNDERSTOOD, THOUGH STUDIES HAVE IMPLICATED A CHROMOSOMAL TRANSLOCATION THAT RESULTS IN A FUSION PROTEIN THAT PROMOTES TUMOR GROWTH THROUGH THE OVERPRODUCTION OF PLATELET-DERIVED GROWTH FACTOR (PDGF). DIAGNOSIS IS MADE VIA SKIN BIOPSY. THIS ACTIVITY DESCRIBES THE EVALUATION, DIAGNOSIS, AND MANAGEMENT OF DERMATOFIBROSARCOMA PROTUBERANS AND STRESSES THE ROLE OF TEAM-BASED INTERPROFESSIONAL CARE FOR AFFECTED PATIENTS.