Dermatofibrosarcoma protuberans

Origin and causes

Dermatofibrosarcoma protuberans (DFSP) is a sarcoma that affects the deep layers of the skin. The majority of DFSP patients are between the ages of 20 and 50. It is most commonly found on the chest followed by the arms, legs, and head and neck. No cause has been identified to date.

 

Symptoms and signs

Symptoms and signs of DFSP often present as a small firm nodule on the skin measuring a few centimeters. It may sometimes be flat or have a purplish or reddish color. This cancer grows very slowly over a period of several months to several years. The vast majority of DFSPs do not metastasize. However, some cases can transform into a much more aggressive cancer called fibrosarcoma.

 

Diagnosis

Diagnosis is often initially suspected after a dermatology consultation. A biopsy in the clinic is necessary to make the diagnosis. Rarely, additional tests such as an MRI are necessary.

 

Treatment

For small DFSPs, Mohs surgeries can be performed by the dermatologist. For larger tumors, a referral to surgery is necessary. Radiation therapy is rarely necessary. For rare inoperable cases, imatinib may be a choice.

 

Follow-up

Follow-up is necessary after surgery to detect recurrences. These are rare and the overall prognosis is excellent.