Dermatofibrosarcoma protuberans: a rare entity and review of the literature

Abstract

Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignant mesenchymal tumor. The incidence of DFSP is 0.1% of all cancers and less than 2% of all soft tissue sarcomas (STS). It can appear at any age, most commonly in individuals aged between 20 and 50 years. The usual location of DFSP is the trunk and it is limited to the dermis. Wide radical excision is the preferred surgical method for therapy of DFSP without distant metastasis. The probability of regional or distant metastases is less than 5%. Patients with positive or close surgical margins have an elevated risk of local recurrence after resection. Adjuvant radiotherapy administered either before or after the surgical treatment reduces the risk of local recurrence.