Dermatofibrosacroma protuberans (DFSP) is a rare, low to intermediate grade and well differentiated sarcoma of mesenchymal tumour. To date, only less than 40 cases reported for vulva DFSP in the literatures.

A 42, Chinese lady presented to our department with bilateral vulva swelling for one year with accelerated growth for the past 1 month. This was associated tenderness and pruritus. On examination, there were 3 ill-defined vulval masses, the largest one measuring 8 x 8 cm on right vulva; another 2 masses was present on the left vulva, each measuring 4 x 4 cm. The mass was firm, non-tender with limited mobility. There were no regional lymph nodes palpable. Doppler ultrasound scan did not reveal any hypervascularity within the solid mass. An inadequate sample was obtained from Fine Needle Aspiration (FNAC). The clinical impression then was vulva and mons pubis lipoma. A wide local excision was performed under general anaesthesia. The procedure was uneventful. The histopathological report showed DFSP with incomplete margins. The tumour cells are diffusely positive for CD34 but negative for pancytokeratin, desmin, SMA, S 100, CD117 and CD99 stains. Ki67 proliferative index is about 20-30%.

DFSP was first reported by Hoffman et al almost nine decades ago. Following that, several reports have been published with the majority involving the body trunk and limb extremities. Other rare locations include scalp, forehead and genital area. Immunochemical staining has shown almost 100% sensitivity in differentiating the tumour from benign condition. As reported, FNAC may not yield a definitive diagnosis. Current treatment modalities include wide local excision, Mohs micrographic surgery and radiotherapy. DFSP has a high recurrence rate ranging from 10-60% even with clear margins of 2 cm. Mohs Surgery is preferred as it allows immediate microscopic examination of entire margin after surgery. Radiotherapy has been used in conjunction with surgical intervention as DFSP is a radiosensitive tumor and most studies have reported that it reduces the rate of recurrence. To date, chemotherapy is not an option of treatment for DFSP and literatures do not support its efficacy. In conclusion, vulva DFMS is extremely rare occurrence. Recognition of this unique clinical entity preoperatively is important for surgical planning to minimize the chance of recurrence.