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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?

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.

For more detailed information, please see the links below.

For more information see:
Liddy Shriver

Dermatofibrosarcoma Protuberans

Genetics Home

Dermatofibrosarcoma protuberans

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