Dermatofibrosarcoma protuberans (DFSP) is very rare, slow growing malignant soft tissue neoplasm that arises from the dermis and has characteristics of asymmetrical infiltration into deeper subcutaneous tissue. They account for around 0.8% to 1 % of all cancers diagnoses each year.

Dermatofibrosarcoma protuberans lesions are mostly seen on the trunk followed by lower extremities, upper extremities, head and neck respectively. DFSP is slightly common on men than in women.

What causes DFSP?

Although the cause is still known, recent laboratory findings have suggested that chromosomal aberrations may contribute to the pathogenesis of DFSP. However no evidence of familial history or hereditary exist in literatures. Several studies agree that injury to the affected skin like surgical scars and site of vaccination may be the predisposing factor.

Clinical presentation:

Although clinical features of DFSP may vary with the stage of disease, DFSP initially may start with a small nipple-like projection or slightly raised asymptomatic papules or plaque like mass on the area of repeated trauma, vaccination site, scar which may likely be ignored. The tumor may gradually enlarge into larger nodular mass.

They are firmly fixed to the overlying skin and not attached to deeper underlying structures, but there may be multiple satellite nodules at the periphery of the main lesion. The tumor is usually skin colored with a brown-yellow or red tinge and pressure on the area of the lesion causes blanching.

Treatment of Dermatofibrosarcoma Protuberans

The treatment of DFSP consists of wide surgical excision with margin control or Mohs micrographic surgery. Radiotherapy may sometimes be used in addition to surgery. Previously tried chemotherapy is ineffective.

Prognosis of DFSP is good. The overall distant metastasis is around 5% and rate of regional metastasis is around 1%. Previously recurrent rate of DFSP were high but with the introduction of Mohs micrographic surgery, the rates have decreased.

1. Rutgers EJ, Kroon BR, Albus-Lutter LE, et al: Dermatofibrosarcoma protuberans: treatment and prognosis. Eur J Surg Oncol 1992; 18:241-248.