Premium
An unusual presentation of dermatofibrosarcoma protuberans with pleomorphic sarcomatous transformation: potential pitfall and diagnostic strategy
Author(s) -
Cesinaro Anna Maria,
Mataca Ema,
Gambini Claudio,
Kutzner Heinz
Publication year - 2016
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/cup.12688
Subject(s) - dermatofibrosarcoma protuberans , pdgfb , pathology , dermatofibrosarcoma , cd34 , undifferentiated pleomorphic sarcoma , sarcoma , factor xiiia , biology , polymerase chain reaction , fusion gene , soft tissue , soft tissue sarcoma , medicine , immunohistochemistry , gene , stem cell , growth factor , biochemistry , genetics , receptor , platelet derived growth factor receptor
Dermatofibrosarcoma protuberans ( DFSP ) is a low grade, superficial sarcoma characterized by a proliferation of monomorphous, spindle cells arranged in a storiform pattern and infiltrating the subcutaneous tissue. The tumor is typically CD34 positive, and shows the characteristic COL1A1‐PDGFB fusion gene, detectable either by florescent in situ hybridization ( FISH ) and polymerase chain reaction ( PCR ). We describe a case of DFSP with a focus of peculiar pleomorphic sarcomatous transformation. The focus constituted the entire bioptic tissue that was initially excised, raising considerable diagnostic problems for pathologist. The use of FISH as an ancillary technique allowed the right diagnosis.