Pigmented dermatofibrosarcoma protuberans. Report of two cases as a variant of dermatofibrosarcoma protuberans with partial neural differentiation.
Two cases of so-called Bednár tumor are reported. The histopathological finding of a typical storiform pattern composed of fusiform tumor cells resembled dermatofibrosarcoma protuberans (DFSP) except for the presence of melanin-containing cells scattered within the lesion. Ultrastructurally, the predominant tumor cells in case 1 possessed extensive cytoplasmic processes, convoluted nuclei, and intercellular junctions, but lacked basal lamina. Melanocytic tumor cells contained melanosomes and were surrounded by basal lamina. Immunohistochemical examinations revealed a positive reaction for S-100 protein and neuron-specific enolase in the pigment-laden cells. Consequently, these two tumors were considered to be a variant of DFSP with partial neural differentiation.
D000328:Adult; D005260:Female; D005354:Fibrosarcoma / Q000378:metabolism / Q000473:pathology*; D006651:Histocytochemistry; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D008544:Melanocytes / Q000473:pathology; D008875:Middle Aged; D010751:Phosphopyruvate Hydratase / Q000378:metabolism; D009418:S100 Proteins / Q000378:metabolism; D012583:Schwann Cells / Q000473:pathology; D012878:Skin Neoplasms / Q000378:metabolism / Q000473:pathology*; D012880:Skin Pigmentation
D009418:S100 Proteins; D010751:Phosphopyruvate Hydratase