Dermatofibrosarcoma Protuberans.

Dermatofibrosarcoma protuberans (DFSP) is a rare superficial soft tissue sarcoma. Its rarity precludes large prospective studies. Clinical diagnosis requires an high index of suspicion. Effective management requires an appreciation of tumor biology and the nature of the characteristic infiltrative growth pattern. DFSP tends to recur locally, with a low risk of dissemination. Aggressive surgical resection with widely negative margins is essential to management. Radiotherapy may be indicated in special circumstances. Understanding the molecular pathogenesis has resulted in use of tyrosine kinase inhibitor therapy for patients with locally advanced disease or in metastatic disease. DFSP patients require long-term follow-up.