Dermatofibrosarcoma protuberans of the vulva: a case report

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a low-grade to intermediate-grade well-differentiated sarcoma of dermal origin. Local recurrence rates are high but distant metastases are rare. This report describes a case of vulvar DFSP requiring four resections for primary clearance. A 56-year-old woman referred after excision of a vulvar tumour and histology had DFSP extending to the resection margins. Two more extended excisions again yielded a specimen with positive margins. The last excision was performed with intraoperative frozen section analysis of the margins. Frozen section analysis of the resected specimen revealed clear margins and this was confirmed by final pathology. The postoperative course was uneventful. The patient has been without recurrence for 15 months. A wide and deep local excision is recommended for both primary and recurrent lesions. The patient had three resections before clear surgical margins were achieved. Intraoperative frozen section analysis is helpful in assessing resection margins.

Figure 1

(A) Residual dermatofibrosarcoma protuberans in the final resection specimen revealed a spindle-cell proliferation within the dermis that infiltrated extensively into the subcutaneous tissues (H&E ×40). (B) Diffuse and strong staining of the spindle-cell proliferation (immunohistochemistry CD34 ×200).