Mohs Micrographic Surgery for Dermatofibrosarcoma Protuberans of the Vulva

Abstract

Introduction. Dermatofibrosarcoma Protuberans (DFSP) is a rare cutaneous tumour of low/intermediate malignant potential, which occasionally arises on the vulva. Historically, the treatment has been wide local excision (WLE). Mohs micrographic surgery (MMS) is now recommended to ensure precise margin control. MMS to treat DFSP of the trunk and extremities is well documented. However, no report to date has described its use in vulval DFSP. Case History. A 39 year old woman presented with a longstanding nodule in the left labium majus. Histology after surgical removal showed an incompletely excised DFSP. MMS was undertaken with primary closure of the defect. Three years following treatment there is no evidence of recurrence. Discussion. The local recurrence rate of DFSP after WLE ranges from 0–75%. Finger-like projections from DFSP into surrounding tissue often results in incomplete excision. Representative vertical sections used in WLE assess less than 1% of the total tumour margin. MMS uses systematic horizontal sectioning. 100% of the tumour margin is microscopically examined. MMS is now advocated to ensure precise margin control.

Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2

“…33 The typical clinical picture consists of a firm plaque with surrounding red to blue discoloration, or less often with multiple small subcutaneous nodules. 34 Histological study shows a storiform pattern of uniform cytologically bland spindle cells, with a characteristic honeycomb pattern of infiltration into the subcutaneous fat (Figure 1). 31,32 Strong positivity for vimentin and CD34 (Figure 2) is revealed by immunohistochemistry.…”

Dermatofibrosarcoma Protuberans of Vulva, A Case Report and Review of Literature

“…The tumor is often solid and due to its indolent nature, often escapes detection in the early stages [21]. It often begins as a small, firm patch of skin that can be reddish, purplish, or even flesh-colored and grows slowly to become a nodule.…”

“…The fibroblasts are elongated and have small nuclei. The cells also display a uniform appearance with little nuclear pleomorphism and low to moderate mitotic activity [21]. DFSP not only infiltrates the dermis, but also the subcutaneous fat and the fascia [6,23].…”

“…The recurrence rates are high after wide local excision, because the tumor often has microscopic projections into the surrounding tissue that extend beyond the central nodule [21]. However, more recent studies have shown that there is a lower recurrence rate with Mohs micrographic surgery (MMS) [35].…”

“…However, more recent studies have shown that there is a lower recurrence rate with Mohs micrographic surgery (MMS) [35]. MMS allows mapping of the tumor along with microscopic examination of the lateral and deep margins [21]. In one study, DFSP treated with MMS had an overall recurrence rate of 1.6%, versus a 20% recurrence rate with wide excision and 43% after conservative excisions [6].…”