A Case of Dermatofibrosarcoma Protuberans in Childhood / 대한피부과학회지

ABSTRACT

Dermatofibrosarcoma protuberans(DFSP) is an uncommon fibrohistiocytic tumor of intermediate malignancy that usually occurs in early or mid-adult life as a nodular cutaneous mass. The tumor occurs most frequently on the trunk or the proximal extremities. A 14-year -old boy was seen for red-brown, hard plaques and nodules on the anterior neck. This asymptomatic lesion had appeared at the age of one year as an erythematous patch and had thereafter slowly enlarged. Recently the lesion grew more rapidly. Biopsy specimens of both the nodule and plaque showed a tumoral proliferation of spindle-shaped cells with a storiform arrangement, being suggestive of DFSP. Immunohistochemical staining for CD34 was positive. The patient was treated with a wide and deep excision. We report herein a case of DFSP in a 14-year -old boy.