Dermatofibrosarcoma Protuberans

Clinical

Wide age range but most common 20-40 years of age
Trunk most common, followed by proximal extremities and head and neck
- Very rare cases of similar appearing tumors in deep tissues and retroperitoneum
  - Same clinical behavior
Slow growth
Early lesions are plaque-like
Late lesions protuberant
10-20% recur if widely excised
- Frequent, aggressive recurrences if not widely excised
Metastases <5% if widely excised
Cases with areas of sarcoma appear to have the same behavior as usual cases if widely excised