Skin Cancer: Back to Basics

Dermatofibrosarcoma protuberans is a soft tissue tumor that arises from the dermis. This relatively rare, low-grade tumor has a high propensity for local recurrence unless it is completely excised. Dermatofibrosarcoma protuberans classically progresses slowly as a plaque on the trunk of young adults. It often resembles a scar that is slowly growing over months to years. Because of its rarity and subtle progression, this form of skin cancer will often go undiagnosed for years. Over time, dermatofibrosarcoma protuberans can become invasive into the subcutaneous fat, muscle, fascia, and chest wall. Diagnosis of dermatofibrosarcoma protuberans is made by a core needle or incisional biopsy. Once diagnosed, cure rates approach 100%. The purpose of this article is to review the epidemiology, pathophysiology, clinical features, differential diagnosis, diagnosis, staging, and treatment of dermatofibrosarcoma protuberans. Increasing healthcare providers' awareness about this rare form of skin cancer and its recognition may improve patient outcomes.