Dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare, locally invasive, slow-growing tumour of the subcutaneous tissue that recurs frequently after surgical excision. Despite its name, DFSP is regarded as a low-grade tumour, however, careful histological evaluation is necessary as a small proportion of lesions develop a high-grade fibrosarcomatous component.

This chapter is set out as follows:

• DFSP has an annual incidence of only 4.2 cases per million
• It tends to present in young to middle-aged adults with a slight female predominance
• There may be a prior history of trauma, burn or previous surgery

Distribution

• The tumour most frequently involves the trunk (42-72%), the proximal extremities (16%-30%), and the head and neck (10-16%) especially the scalp

Morphology

• The initial presentation of DFSP is a single, raised, red to bluish, firm, cutaneous nodule or plaque
• Overlying the tumour the skin surface can be brown-yellow, red-tinged, sclerodermiform or telangiectatic 
• Lesions grow very slowly to form irregular, protuberant swellings 
• DFSP is extremely infiltrative and has a locally destructive growth pattern that can invade the underlying structures such as fascia, muscle and bone
• At advanced stages, the tumour can ulcerate, bleed and become painful

• Management is in Secondary Care, treatment is with a wide local excision of between 2-4 cm, or Mohs micrographic surgery 

• Local recurrence is not uncommon, and can occur many years later. Metastatic spread is uncommon, and is perhaps confined to those tumours that have developed a high-grade fibrosarcomatous component