Outcomes of surgery for dermatofibrosarcoma protuberans
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.
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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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