Congenital dermatofibrosarcoma protuberans: Variability in presentation

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

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

72 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)207-211
Number of pages5
JournalArchives of Dermatology
Issue number2
StatePublished - Feb 1 2003

ASJC Scopus subject areas

  • Dermatology


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