Advances in management of dermatofibrosarcoma protuberans

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare soft-tissue tumor that most commonly presents on the trunk and extremities of adults. It is characterized by low metastatic potential and a favorable prognosis, but extensive subclinical growth can contribute to a high risk of local recurrence. Surgical excision is the first-line treatment, using Mohs micrographic surgery or wide local excision with careful evaluation of the peripheral and deep surgical margins. Adjuvant therapy may be beneficial in patients with unresectable, recurrent, or metastatic DFSP. Historically, adjuvant radiation therapy has been used to reduce the risk of local recurrence when residual disease is present after surgery. The advent of targeted molecular therapies, such as the selective tyrosine kinase inhibitor, imatinib mesylate, has provided new effective and safe options for adjuvant treatment of DFSP.