Dermatofibrosarcoma Protuberans, a Challenging Entity – Case Report and Brief Literature Review

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive soft tissue tumour of mesenchymal origin, commonly arising in the dermis and extending into deeper tissues. This article presents a case of a 52-year-old male with recurrent DFSP affecting the posterior neck region. Initial surgical excision in 2016 revealed a spindle-cell tumour with storiform patterns and a low mitotic index, while recurrence in 2022 exhibited a multinodular, infiltrative mass with increased cellular atypia and a high mitotic rate. Immunohistochemistry confirmed diffuse CD34 positivity in both instances. Complete surgical excision, including re-excision for margin involvement, achieved tumour-free status. The case highlights the importance of histological evaluation, surgical approach and follow-up in these cases. Long-term monitoring remains essential due to DFSP’s high recurrence potential.