title: A study on the prognostic value of clinical and surgical features of dermatofibrosarcoma protuberans in Korean patients.
abstract: BACKGROUND
  Dermatofibrosarcoma protuberans (DFSP) is a rare spindle cell tumour with locally aggressive characteristics. Only few studies on the epidemiology of DFSP in Asians have been reported.
  
  
  OBJECTIVE
  : The purpose of the study was to evaluate the epidemiological and clinical characteristics of DFSP in Korean patients and determine the prognostic factors that affect disease-free survival.
  
  
  METHODS
  We conducted a retrospective review of patients diagnosed with primary or recurrent DFSP between 2000 and 2009 at three Seoul National University Hospitals. Patient, tumour and treatment factors were analysed for local recurrence-free survival.
  
  
  RESULTS
  We analysed data for 65 patients, of whom 36 (55.4%) were female. The mean age at disease onset was 34.4 years with 50% aged between 20 and 40 years. Involved sites were most often on the trunk (66.1%). Among the 65 patients, 60 patients underwent wide excision; seven patients (10.8%) experienced local recurrence during 3.6 years of follow-up. The recurrence-free survival was significantly related to the microscopic resection margins (P = 0.005), clinical presentation (P = 0.002) and frequency of recurrence (P = 0.014) in the univariate analysis. There were three cases with metastasis, two of whom died because of progression of the disease despite continuous chemotherapy with imatinib mesylate.
  
  
  CONCLUSIONS
  We report the epidemiologic and clinical characteristics of DFSP in Korea. Patients with recurrent disease at presentation and frequent recurrence have a high risk of recurrence and metastasis, and therefore close observation is needed in these patients.
authors.affiliation: Department of Dermatology, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: M
authors.initials: M
authors.lastname: Kim
authors.forename: C H
authors.initials: CH
authors.lastname: Huh
authors.forename: K H
authors.initials: KH
authors.lastname: Cho
authors.forename: S
authors.initials: S
authors.lastname: Cho
mesh_terms: D000328:Adult; D018223:Dermatofibrosarcoma / Q000473:pathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D011379:Prognosis; D012008:Recurrence; D056910:Republic of Korea