Dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans is a soft tissue sarcoma that develops in the dermis. It is rarely bumpy and metastases occur in only a few cases. However, sarcoma usually occurs between the ages of 20 and 50 years, but in rarer cases it can be seen even in infancy.

The disease typically manifests itself as indurated purple or pink plaques or nodular masses on the head, neck, trunk or extremities. The disease is caused by a chromosomal translocation at the loci t(17; 22).

Of all skin sarcomas, dermafibrosarcoma protuberans is the most common one.