Detection of Dermatofibrosarcoma Protuberans and Giant Cell Fibroblastoma

8 Molecular Detection of Dermatofibrosarcoma Protuberans and Giant Cell Fibroblastoma 

Dermatofibrosarcoma protuberans and its pediatric counterpart giant cell fibroblastoma are two tumors sharing many histological, immunohistochemical and cytogenetic features. 

Dermatofibrosarcoma protuberans is a slow infiltrative growing fibrohistiocytic tumor mainly affecting adults between the 3 and 4 decade and appears in the deep dermis, composed of proliferating uniform small spindled cells arranged in a repetitive storiform pattern. 

Giant cell fibroblastoma is a rare pediatric dermal or subcutaneous slowly growing tumor with a predilection for children under 10 years of age. Histologically the tumor consists of proliferating spindled cells mixed with multinudeated giant cells surrounded by solid fibromyxoid stroma dissected by vascular like spaces. 

Dermatofibrosarcoma protuberans and giant cell fibroblastoma share the same immunohistochemical profile and show positive reaction with the specific antibodies to CD34, PDGF and vimentin. Both tumors reveal low or intermediate malignant behavior and tens to local recurrence after incomplete excision and characterized by very low metastatic tendency, which generally occurs after an inadequate surgical treatment. 

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