Dermatofibrosarcoma protuberans in children.

Dermatofibrosarcoma protuberans (DFSP) is a relatively rare neoplasm affecting the skin. It is an infiltrative tumour of intermediate malignancy, with a limited potential for metastasis but a high rate of recurrence. The incidence in children is even less frequent, although a proportion of those identified in adulthood may reflect a delay in diagnosis of childhood DFSP. We report the experience of DFSP seen at The Children's Hospital at Westmead (Sydney, Australia). Three children aged 5, 10 and 11 years of age underwent surgical excision of their lesions. Recurrence was evident in one child whose initial histopathology was not definitive for DFSP, and whose initial surgery had not involved wide local excision. All three children were male, and all had lesions affecting their trunk. One child whose lesion was thought to have been evident since birth may have represented congenital DFSP.