DOI: 10.36347/sasjs.2021.v07i10.011

Pages: 572-575

Dermatofibrosarcoma protuberans (DFSP) is a rare tumour of the soft tissue tumor involving the dermis, subcutaneous tissue, and in few cases, muscle and fascia. The tumour typically presents as a slowly growing, firm plaque or nodular on the trunk and extremities of young adults. The etiology of dermatofibrosarcoma protuberans has been attributed to a chromosomal translocation t (17;22)(q22;q13) that results in a fusion protein promoting tumour growth through the overproduction of platelet-derived growth factor (PDGF). Diagnosis is made via skin biopsy. Treatment options include complete surgical excision by performing conventional surgery with wide margins (>3 cm) or Mohs micrographic surgery. These lesions are locally aggressive with high rate of recurrence following surgery but the prognosis is excellent when it is treated effectively. These tumours have intermediate malignant potential.