Outcomes of surgery for dermatofibrosarcoma protuberans

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Abstract

Background. Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma usually presenting as nodular cutaneous mass on the trunk and proximal extremity. The tumour grows slowly, typically over years. The standard treatment is wide local excision with at least a 3-cm margin. The local regional recurrence is up to 50%, emphasizing the need for wide margins for local control. A small fraction of DFSP may metastasize, but on histological examination such tumours have features of fibrosarcomas rather than DFSP.

Hypothesis. This study was done to review our experience of the time interval to recurrence of DFSP.

Design. A retrospective review was undertaken to identify patients with DFSP in our university teaching hospital.

Methods. All patients received their primary surgical treatment in our department between February 1968 and June 2001. Treatment consisted of wide local excision with margins of at least 3 cm. The chi-square test and Fisher's exact test were performed to determine the relationship between recurrence and clinicopathological variables. We evaluated the prognostic variables using the Kaplan–Meier method with log-rank comparison.

Results. The median follow-up period was 59 months. The 5 and 10-year disease-free survival (DFS) were 86 and 76%, respectively. The overall recurrence rate was 16.7%. The mean time to recurrence was 38±12 months (range 1–100 months). In 30% of those patients with recurrences, the local regional recurrence was after 5 years.

Conclusion. Wide local excision with good margins decreases local regional recurrences in patients with DFSP. Close surveillance is necessary even beyond 5 years because late recurrences occur.

Introduction

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma usually presenting as nodular cutaneous mass, representing some 1% of all sarcomas.1 DFSP commonly presents on the trunk and extremities, between the second and fifth decades of life. Treatment is wide surgical resection.2 However, local regional recurrences between 24 and 60% have been reported.3 A small fraction of DFSP may metastasize, but these may be typical fibrosarcomas rather than DFSP.4 We sought to analyse disease-free survival and time interval to recurrence of DFSP patients who were managed in our department.

Section snippets

Methods

We identified 60 patients with DFSP at the University of Illinois at Chicago, Department of Surgical Oncology registry from, February 1968, to, June 2001. Each year the department treats approximately 100 patients with soft tissue sarcoma. Surgical treatment consisted of wide local excision with at least a 3-cm margin incorporating the underlying deep fascia and overlying skin. Adjuvant radiation therapy was primarily given to patients with margins and/or patients who refused further surgical

Patient and tumour characteristics

Sixty patients underwent surgical treatment for DFSP during the study period. There were 23 male and 37 female patients. Mean age was 36±14 years (range 10–70 years). The location of the primary tumour was the trunk in 23, the head and neck in 16, upper extremity in 11, and the lower extremity in 10. Three patients received post-operative radiation. Mean margin was 3.1 cm (range 1–6 cm) in which that data was available. Fifty-six patients had tumours 5 cm or less in size (Table 1).

Fifty-seven

Discussion

DFSP is an intermediate neoplasm composed of fibrohistiocytic cell that may entrap subcutaneous adipocytes diffusely or grows in stratified horizontal plates in the subcutaneous fat.5 The microscopic appearance of DFSP consists of monomorphous spindle cells arranged in a storiform pattern and embedded in a sparse to moderately dense fibrous stroma. Immunohistochemistry with factor XIIIa and CD34 may help differentiate between dermatofibroma and DFSP. Dermatofibroma is factor XIIIa-reactive and

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