A 32 year-old woman presents with an enlarging bump on her wrist. She first noticed it about two years ago as a small, reddish-purple bump. She was told by a healthcare provider at a drug store “rapid clinic” that it was a harmless lesion called a dermatofibroma. Over the last year, it has grown to the point that it interferes with wearing jewelry.
What’s your diagnosis?
The lesion was biopsied and found to be Dermatofibrosarcoma Protuberans.
Dermatofibrosarcoma Protuberans (DFSP) is a low-grade, soft tissue sarcoma of the skin. It most commonly presents between ages 20 and 50. A DFSP most commonly occurs on the trunk, but they may be found anywhere, as shown in this case. Initially, a DFSP can start out as a small papule or even an indurated, depressed area that can mimic morphea. Eventually, the lesion evolves into a nodular form. It is typically asymptomatic. A DFSP has an aggressive growth pattern, often invading underlying muscle and bone. It has a significant rate of both recurrence and metastases.
In 2006, the FDA granted approval for imatinib mesylate (Gleevec) as a single agent for the treatment of DFSP in adult patients with a recurrent, metastatic, or unresectable DFSP. The current thinking is that Gleevec may also be used as a pre-surgical/concomitant therapy with Moh’s surgery (especially when a DFSP is on the head or neck) or with deep aggressive excisions with wide margins.
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