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. OBJECTIVES : 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.