title: Dermatofibrosarcoma protuberans with areas of giant cell fibroblastoma in the vulva: a case report.
abstract: OBJECTIVE
  To review the clinical, morphologic, immunohistochemical, and histogenetic characteristics of dermatofibrosarcoma protuberans with areas of giant cell fibroblastoma and explore current treatment options.
  
  
  METHODS
  We describe the case of a 38-year-old patient with a tumor measuring 5.7 cm on the right labium majus of the vulva. Serial sections stained with hematoxylin-eosin were examined and immunohistochemical staining was performed for CD34 and PDGF receptor alpha and beta (PDFGRA and PDGFRB).
  
  
  RESULTS
  The histologic study showed spindle-cell proliferation typical of dermatofibrosarcoma protuberans and other areas containing fibrosis and giant cells lining pseudovascular spaces. Both tumor areas expressed CD34, PDGFRA, and PDGFRB.
  
  
  CONCLUSIONS
  Only two cases of dermatofibrosarcoma protuberans with areas of giant cell fibroblastoma in the vulva have been reported to date. Both dermatofibrosarcoma protuberans and giant cell fibroblastoma are characterized by the translocation t (17;22) (q22;q13). The fact that PDGFRA and PDGFRB are overexpressed in these tumors opens new treatment options with imatinib. Surgical excision with wide margins or Mohs micrographic surgery continues to be the treatment of choice.
authors.affiliation: Department of Pathology, Hospital Universitario Ramón y Cajal, Madrid, Spain. apascualm.hrc@salud.madrid.org
authors.forename: A
authors.initials: A
authors.lastname: Pascual
authors.forename: C
authors.initials: C
authors.lastname: Sánchez-Martínez
authors.forename: C
authors.initials: C
authors.lastname: Moreno
authors.forename: A
authors.initials: A
authors.lastname: Burdaspal-Moratilla
authors.forename: M J
authors.initials: MJ
authors.lastname: López-Rodriguez
authors.forename: L
authors.initials: L
authors.lastname: Rios
mesh_terms: D000328:Adult; D018952:Antigens, CD34 / Q000032:analysis; D001706:Biopsy; D018223:Dermatofibrosarcoma / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D005260:Female; D005870:Giant Cell Tumors / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D006801:Humans; D007150:Immunohistochemistry; D010982:Platelet-Derived Growth Factor / Q000032:analysis; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D016896:Treatment Outcome; D014846:Vulvar Neoplasms / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology / Q000601:surgery
chemical_list: D018952:Antigens, CD34; D010982:Platelet-Derived Growth Factor; C075278:platelet-derived growth factor A