Case Report - Childhood onset dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow growing, locally aggressive dermal tumor of disputed histogenesis with a tendency for local recurrence. A 16-year-old female presented with hyperpigmented macules, papules, plaques, erythematous nodules and atrophic, indurated lesions ranging from a few millimeters to a few centimeters. Biopsy revealed thinned out epidermis and a diffuse dermal infiltration by fascicles of spindle cells arranged in storiform pattern. The pseudoseptae formed by the tumor cells interdigitating between the subcutaneous adipocytes resulted in a honeycomb pattern. Histochemical findings are helpful, but the chronicity, size, indolent nature, recurrence after surgery and histopathology are more important to arrive at a diagnosis. Wide and deep surgical excision is the treatment of choice.