title: A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change.
abstract: BACKGROUND
  To our knowledge, no systematic review of dermatofibrosarcoma protuberans (DFSP) outcomes based on the presence or absence of fibrosarcomatous (FS) change has been performed.
  
  
  OBJECTIVE
  We sought to compare available outcome data for DFSP versus DFSP-FS.
  
  
  METHODS
  The literature was searched for DFSP and DFSP-FS reports with outcome data (local recurrence, metastasis, or death from disease). Chi-square tests were calculated to determine whether DFSP and DFSP-FS significantly differed in risk of local recurrence, metastasis, and death from disease.
  
  
  RESULTS
  In all, 24 reports containing 1422 patients with DFSP and 225 with DFSP-FS are summarized. Risk of local recurrence, metastasis, and death from disease in DFSP-FS was significantly higher as compared with DFSP (local recurrence 29.8% vs 13.7%, risk ratio 2.2 [95% confidence interval 1.7-2.9]; metastasis 14.4% vs 1.1%, risk ratio 5.5 [95% confidence interval 4.3-7.0]; and death from disease 14.7% vs 0.8%, risk ratio 6.2 [95% confidence interval 5.0-7.8]). There was no significant difference in DFSP-FS outcomes based on proportion of FS change within tumors.
  
  
  CONCLUSIONS
  This study is based on previously reported data from different hospitals with no uniform process for reporting FS change. The impact of confounders (age, immune status, tumor location, treatment) could not be evaluated because of limited data.
  
  
  CONCLUSIONS
  Based on available retrospective data, risk of metastasis and death is elevated in DFSP-FS as compared with DFSP. Even a low degree of FS involvement portends worse outcomes.
authors.affiliation: Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
authors.forename: Christine A
authors.initials: CA
authors.lastname: Liang
authors.affiliation: Department of Dermatology, Mayo Clinic, Jacksonville, Florida.
authors.forename: Anokhi
authors.initials: A
authors.lastname: Jambusaria-Pahlajani
authors.affiliation: Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
authors.forename: Pritesh S
authors.initials: PS
authors.lastname: Karia
authors.affiliation: Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Rosalie
authors.initials: R
authors.lastname: Elenitsas
authors.affiliation: Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Paul D
authors.initials: PD
authors.lastname: Zhang
authors.affiliation: Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. Electronic address: cschmults@partners.org.
authors.forename: Chrysalyne D
authors.initials: CD
authors.lastname: Schmults
mesh_terms: D001707:Biopsy, Needle; D018223:Dermatofibrosarcoma / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D005260:Female; D005354:Fibrosarcoma / Q000401:mortality / Q000473:pathology / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D009361:Neoplasm Invasiveness / Q000473:pathology; D009364:Neoplasm Recurrence, Local / Q000401:mortality / Q000473:pathology*; D009367:Neoplasm Staging; D011379:Prognosis; D018570:Risk Assessment; D012878:Skin Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D016019:Survival Analysis
keywords: dermatofibrosarcoma protuberans; dermatofibrosarcoma with fibrosarcomatous change; fibrosarcomatous dermatofibrosarcoma protuberans