title: Sclerosing dermatofibrosarcoma protuberans.
abstract: Dermatofibrosarcoma protuberans is a low-grade sarcoma of the skin with well characterized clinical and histopathological features. The lesion frequently appears as a plaque on the trunk or on the extremities of middle-aged adults. Microscopically, the tumor is composed of monomorphous spindle cells arranged in storiform pattern and embedded in a sparse to moderately dense fibrous stroma. We report on two unusual cases of dermatofibrosarcoma protuberans in which neoplastic cells were diffusely replaced by sclerotic tissue in more than 50% of the entire tumor. In both cases, no external trauma or radiotherapy were recorded. Since tumor regression has been defined as a loss of tumor mass in the absence of any treatment (or trauma) we believe that sclerosis in DFSP may represent a manifestation of regression and not, as previously suggested, a new variant of the neoplasm.
authors.affiliation: Center for Dermatopathology, Freiburg, Germany.
authors.forename: C
authors.initials: C
authors.lastname: Diaz-Cascajo
authors.forename: W
authors.initials: W
authors.lastname: Weyers
authors.forename: S
authors.initials: S
authors.lastname: Borghi
mesh_terms: D000368:Aged; D018952:Antigens, CD34 / Q000032:analysis; D018223:Dermatofibrosarcoma / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008875:Middle Aged; D012598:Sclerosis; D012867:Skin / Q000737:chemistry / Q000473:pathology*; D012878:Skin Neoplasms / Q000378:metabolism / Q000473:pathology*; D014746:Vimentin / Q000032:analysis
chemical_list: D018952:Antigens, CD34; D014746:Vimentin