Korean J Dermatol.  2010 Jul;48(7):624-626.

A Case of Congenital Dermatofibrosarcoma Protuberans

• Kim MS
• Han TY
• Lee JH
• Son SJ

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a tumor of intermediate malignancy that has long been considered to almost exclusively affect adults. Congenital DFSP is extremely rare and the diagnosis is often difficult because of the deceptive appearance of the lesions. A 12-month-old infant developed an asymptomatic red indurated nodule on the lower aspect of his back. The brownish macule had appeared at birth and the lesion grew rapidly 6 months later. Histologic examination showed spindle shaped cells with a storiform pattern and the cells were positive for CD34 stain. A diagnosis of congenital DFSP was made and the mass was removed by wide local excision. There was no evidence of recurrence at follow-up 8 months later.

Keyword

Congenital dermatofibrosarcoma protuberans

MeSH Terms

Adult
Dermatofibrosarcoma
Follow-Up Studies
Humans
Infant
Parturition
Recurrence
Dermatofibrosarcoma