Dermatofibrosarcoma Protuberans

Key Points
Rare type of skin cancer
Develops in second layer of skin
Growths in skin enlarge into masses

Dermatofibrosarcoma Protuberans is a very rare type of skin cancer (it only affects one in 10 million people) that develops in the dermis, or second layer of skin. The cause is not entirely known, but has something to do with the body’s rearrangement of genes (chromosomal translocation), which causes it to produce a protein called kinase, which triggers the growth of Dermatofibrosarcoma Protuberans (DFSP). Those growths in the skin eventually grow into bulging masses, which may become tender and bleed. DFSP is most often found on the trunk, arms. legs, and the head and neck. It usually does not spread to other parts of the body, however, it may grow very aggressively in the skin.

Differential Diagnosis (Other conditions with similar appearance)

Dermatofibroma
Epidermal Inclusion Cyst
Keloid and Hypertrophic scar
Malignant Melanoma
Metastatic Carcinoma of the Skin
Morphea

Diagnosis
Blood tests are not available. Diagnosis is determined by skin biopsy.

Treatment
Surgery
*Radiation or Chemotherapy in some cases

Surgery is the standard procedure to rid the body of DFSP. Unfortunately, because the disease grows aggressively and covers a large part of the body, surgery often requires the removal of not only the DFSP, but a large amount of the surrounding skin, which helps the disease from reoccurring. However, MOHS surgery is the most effective type of surgery — it will remove the tumor and all related cells without a wide-area excision. Nevertheless, re-occurrence rates are still high for DFSP after treatment.

Where DFSP spreads to other parts of the body, it is potentially fatal. Fortunately, the disease itself is rare, and it’s doubly rare for DFSP to spread to other parts of the body.