Dermatofibrosarcoma protuberans—a review of 10-year experience on wide local excision

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma of low to intermediate grade. Correct diagnosis is very often difficult to make on initial presentation. It is a locally destructive tumour which seldom metastasizes. Excision with an adequate margin is the recommended treatment. However, local recurrence is not uncommon with ranges from 4% to 21% in the literature. Medical records of all patients suffering from DFSP who received operation in our centre were reviewed. The age, gender, location of tumour and method of wound closure were described. Factors that may contribute to tumour recurrence were analysed. Between January 1997 and December 2007, 32 patients (20 men) with DFSP, age ranging between 15 and 78 years, were treated. The lesions (22, 69%) located on the trunk and the lesions (five, 15.5%) located on the extremities were resected with a 3-cm margin, while those located in the head and neck region (5, 15.5%) were excised with a 1.5-cm margin. Intra-operative frozen section was performed in all cases. Final histological examination showed 30 cases of classic DFSP and two cases of DFSP with fibrosarcomatous transformation (FS-DFSP). The positive margin rate was 3.2%. In all cases, tumour resection and reconstruction were performed in a single-stage operation. Adjuvant therapy was given to two cases of FS-DFSP and one case of involved resection margin. With a mean follow-up of 51 months, there was no local recurrence in the classic DFSP group, while the two patients with FS-DFSP developed local recurrence at post-operative 4 and 10 months, respectively. We echo the literature that wide local excision with clear margins is the recommended treatment for the classic DFSP. The achievement of low positive margin rate is crucial for a single-stage operation and to minimise the use of adjuvant therapy.