Dermatofibrosarcoma protuberans: a clinicopathological analysis of 10 cases in Asians

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive cutaneous neoplasm that exhibits a marked tendency for recurrence after local excision. This case series aims to study the clinical, histological and immunohistochemical features of DFSP in Asians. Ten patients with DFSP diagnosed between 1992 and 2001 were reviewed. There were more women than men in a ratio of 4:1. There were six Chinese, two Malays, one Indian and one Eurasian. The mean age was 38 years. The duration of each lesion before excision varied from 6 months to 27 years. Fifty per cent of tumours occurred on the trunk. On histology, all the lesions were dermal-centred spindle cell tumours, extending to the subcutis, and exhibited the characteristic storiform pattern. One tumour also demonstrated fibrosarcomatous changes. Two tumours were of the rare pigmented variant (Bednar tumour). Immunohistochemistry with CD34 was positive in all cases, except the fibrosarcomatous area of one tumour, which was negative for CD34. For comparison, six cases of deep-penetrating dermatofibroma were stained for CD34 and all showed an absence of CD34 expression. Wide excision of the tumour was performed in all cases of DFSP. There was no recurrence after mean follow up of 6 years (range 2.25-9.5 years).