Dermatofibrosarcoma protuberans: symptoms, diagnosis and treatment

Summary. Dermatofibrosarkoma protuberans (DFSP) is characterized by a high frequency of recurrence and a low risk of metastasis. The mass fraction DFSP is 1% of soft tissue sarcomas. The disease is most common in patients 20–50 years old and mainly localized on the trunk, especially on the chest wall and the shoulder girdle; on the genetic level the cause of DFSP is the mutual translocation of t(17;22)(q22; q13). The unfavorable prognostic factors for DFSP is the advanced age of patients, the tumor size more than 5 cm, the location on the area of head and neck, the high mitotic index, p53 mutation and the increased number of cells in the histological preperation. The most effective method of surgery treatment is microscopically controlled operation for Mohs, which leads to the smallest number of relapses. As an adjuvant treatment of positive edges of the resection can be used radiotherapy by the total focal dose 50–60 Gy. With the local widespread and the metastatic DFSP is using the inhibitor of the tyrosine kinase imatinib.