Dermatofibrosarcoma protuberans : a report of 36 cases in Hong Kong and review of the literature 36

Objective: To report a case series of dermatofibrosarcoma protuberans focusing on the clinical presentation and prognosis of this tumour. Patients and Methods: A total of 36 patients with confirmed histological diagnosis of dermatofibrosarcoma protuberans were retrieved from the database of Queen Elizabeth Hospital, Hong Kong from 1994 to 2007. Their demographic characteristics, clinical manifestations, management and clinical outcomes were documented and tabulated. Results: Up to 86% of cases occurred in adults between 30 and 60 years old without gender predilection. Most of them showed delayed presentation with 90% and 60% of cases presenting at least 6 months and 1 year prior to the diagnosis of dermatofibrosarcoma protuberans respectively. Asymptomatic growth was the leading clinical manifestation. The most prevalent sites were trunk followed by extremities and then head and neck region. In our series, all patients received surgical treatment, with clear margin achieved in 25 patients (69.4%) but the margin was involved in the remaining 11 patients (30.6%). Post operative radiotherapy and chemotherapy were offered to 9 patients (25.0%) and 3 patients (8.3%) respectively. Metastases occurred in 5 patients (13.9%). Conclusion: In spite of its deceptively benign clinical manifestation, dermatofibrosarcoma protuberans should draw the attention of dermatologists for its variability and the extent of excision could be reduced by early detection.