Dermatofibrosarcoma protuberans (DFSP). A narrative review of the literature and current guidelines for its management.

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a slow growing low grade malignant tumour with a high rate of local recurrence and lower rate of metastasis and effecting patients between the 3^rd and 5^th decade. It accounts for less than 1% of all soft tissue sarcomas and can equally involve the trunk and extremities. It is usually presents in the subcutaneous tissue but can grow deeper if increases in size. Diagnosis is usually with biopsy and is confirmed with its typical histological appearance of uniform spindle cells arranged in a storiform or cartwheel-like pattern with minimal cytologic atypia and low mitotic activity and shows diffuse positivity for CD34 on immunohistochemistry. The standard treatment after staging involves wide excision to achieve clear margins hence avoiding risk of local recurrence, which may be reduced with the use of adjuvant radiotherapy (RT), however, its use should be considered after discussion in multidisciplinary team meetings for select cases. The use of RT is more pronounced in larger/ unresectable or metastatic disease cases along with the use of Tyrosine kinase inhibitors (TKI) which has shown very promising results, particularly with Imatinib in cases with PDGFB mutations. The reported overall survival at 10 years following appropriate surgical treatment with localized DFSP is around 90%. The aim of this current review is to highlight the clinical features of DFSP, its surgical management and adjuvant treatments along with the current management guidelines.