title: Vascular congenital dermatofibrosarcoma protuberans:  a new histological variant of dermatofibrosarcoma protuberans.
abstract: We describe a case of congenital dermatofibrosarcoma protuberans (DFSP) that masqueraded as a vascular tumor both clinically and histologically. Based on the infiltrative growth pattern, presence of capillary-sized vessels, and spindle cell areas with slit-like vascular spaces and numerous thin-walled vessels at the periphery of the tumor, a kaposiform hemangioendothelioma was initially diagnosed. Strong diffuse CD34 positivity and the extension into the subcutaneous fat with a sieve-like effect prompted the fluorescence in situ hybridization analysis, which demonstrated a reciprocal t(17;22) translocation. According to our knowledge, this is the first report of a vascular histological variant of DFSP. This unique variant represents a potential pitfall for dermatopathologists and underlines the importance of cytogenetic diagnostics in unusual cases of DFSP.
authors.affiliation: Department of Dermatology, University of Cologne, Cologne, Germany. iliana.tantcheva-poor@uk-koeln.de
authors.forename: Iliana
authors.initials: I
authors.lastname: Tantcheva-Poor
authors.forename: Nicos
authors.initials: N
authors.lastname: Marathovouniotis
authors.forename: Heinz
authors.initials: H
authors.lastname: Kutzner
authors.forename: Thomas
authors.initials: T
authors.lastname: Mentzel
mesh_terms: D018952:Antigens, CD34 / Q000032:analysis; D014408:Biomarkers, Tumor / Q000032:analysis; D002196:Capillaries / Q000737:chemistry / Q000473:pathology; D002886:Chromosomes, Human, Pair 17; D002892:Chromosomes, Human, Pair 22; D018223:Dermatofibrosarcoma / Q000145:classification* / Q000151:congenital / Q000235:genetics / Q000473:pathology / Q000601:surgery; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D007223:Infant; D008297:Male; D011237:Predictive Value of Tests; D012878:Skin Neoplasms / Q000145:classification* / Q000151:congenital / Q000235:genetics / Q000473:pathology / Q000601:surgery; D014178:Translocation, Genetic; D016896:Treatment Outcome
chemical_list: D018952:Antigens, CD34; D014408:Biomarkers, Tumor