John Libbey Eurotext

European Journal of Dermatology


Two childhood cases of pigmented dermatofibrosarcoma protuberans with atrophic change Volume 28, numéro 2, March-April 2018


  • Figure 1
Department of Pathology, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China

Dermatofibrosarcoma protuberans (DFSP) is an extremely rare, locally aggressive mesenchymal neoplasm of the skin, characterized clinically by a protuberant plaque or nodule, and histopathologically by a monomorphous population of spindle cells arranged in a storiform or cartwheel pattern. The pathogenesis of DFSP still remains unclear, and over 90% DFSPs are genetically characterized by the translocation t(17;22)(q22;q13), resulting in the COL1A1/PDGFB fusion gene [1]. DFSP commonly occurs in patients [...]