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Variability in Presentation

Jill M. Weinstein, BS; Beth A. Drolet, MD; Nancy B. Esterly, MD; Maureen Rogers, MD; Bruce S. Bauer, MD; Annette M. Wagner, MD; Anthony J. Mancini, MD

Arch Dermatol. 2003;139:207-211.

Background  Dermatofibrosarcoma protuberans (DFSP) is an uncommon low-grade fibrohistiocytic tumor that usually occurs on the trunk or proximal extremities and typically appears during the second to fifth decade of life. It most commonly begins as a red-blue plaque that grows slowly and ultimately becomes nodular. The tumor is associated with a high recurrence rate but low metastatic potential. It rarely presents in childhood and is even more rarely present at birth. The clinical diagnosis of DFSP in infancy or childhood may be difficult because, in its early stages, the tumor often resembles a vascular birthmark.

Observations  We studied 6 patients with congenital DFSP who were initially thought to have other diagnoses, highlighting the potential clinical variability in presentation. Half of the cases in this series occurred in areas of the body outside of the typically reported distribution pattern of acquired DFSP and in locations that, therefore, may not arouse suspicion of congenital DFSP.

Conclusions  Given the aggressive local potential and high recurrence rate of DFSP, early diagnosis is preferable to facilitate appropriate excision. We recommend that any infant or child presenting with a cutaneous plaque or nodule, even congenital, that does not have characteristic or diagnostic clinical features undergo tissue biopsy for histologic evaluation.

From the Departments of Pediatric Dermatology (Ms Weinstein and Drs Wagner and Mancini) and Plastic Surgery (Dr Bauer), Northwestern University Feinberg School of Medicine, and Division of Dermatology, Children's Memorial Hospital, Northwestern University Feinberg School of Medicine (Drs Wagner and Mancini), Chicago, Ill; Department of Pediatric Dermatology, Medical College of Wisconsin and Children's Hospital of Wisconsin, Milwaukee (Drs Drolet and Esterly); and Department of Pediatric Dermatology, Royal Alexandra Hospital for Children, Sydney, New South Wales, Australia (Dr Rogers).

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