Dermatofibrosarcoma protuberans at the site of a central venous line. Case report

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive cutaneous tumor that usually does not metastasize. Clinically, it may present as a reddish macule to protuberant nodule affecting the trunk and proximal extremities of young and middle-aged adults. Histologically, it is characterized by a monomorphous storiform proliferation of spindle cells, involving the dermis and hypodermis, with a honeycomb pattern of infiltration of the subcutaneous fat. In case of difficulty in diagnosis, immunohistochemical markers (especially CD34) are highly sensitive for DFSP. It is important to remember that the rarity of DFSP, its resemblance to hypertrophied scars and keloids, and its appearance at trauma sites sometimes make the diagnosis of DFSP difficult and delayed. In the literature, DSFP was not described in a child until 1957. Here we report an instance of DFSP at an unusual site in a 9-year-old child, which was the location of a previous central venous line insertion in the left supraclavicular area. A complete excision of the tumor with a wide surgical margin of 3cm of visibly uninvolved tissue was performed, followed by a deltopectoral flap and skin split graft. Postoperatively, the patient's general condition was stable and he was discharged after 5 days to be followed up 3 months later in our clinic.