title: Dermatofibrosarcoma protuberans arising in the context of Shwachman-Diamond syndrome.
abstract: Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignant spindle-cell tumor usually presenting in adulthood. The epidemiology of DFSP has recently been reviewed, and there have been 152 reported cases of DFSP in patients below the age of sixteen. We present the case of a DFSP arising in a young patient with Shwachman-Diamond syndrome (SDS).
authors.affiliation: Department of Dermatology, Boston University Medical Center, Boston, Massachusetts, USA.
authors.forename: Jason E
authors.initials: JE
authors.lastname: Sack
authors.forename: Louis
authors.initials: L
authors.lastname: Kuchnir
authors.forename: Marie-France
authors.initials: MF
authors.lastname: Demierre
mesh_terms: D001706:Biopsy; D001855:Bone Marrow Diseases / Q000150:complications*; D002277:Carcinoma / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D018223:Dermatofibrosarcoma / Q000209:etiology*; D010188:Exocrine Pancreatic Insufficiency / Q000150:complications*; D005260:Female; D006801:Humans; D008068:Lipomatosis / Q000150:complications*; D000081003:Shwachman-Diamond Syndrome; D012878:Skin Neoplasms / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D055815:Young Adult