A case of epithelioid sarcoma arising in the vulva

Abstract

Epithelioid sarcoma of vulva is extremely rare. Only 20 cases have been reported in the literature so far. Early diagnosis is difficult because of its benign appearance as a painless subcutaneous nodule. Therefore treatment can be delayed, although epithelioid sarcoma requires prompt surgery. We report a case of epithelioid sarcoma that occurred in the left vulva with a brief review of literature.

Proximal-type epithelioid sarcoma—unusual presentation: unilateral vulvar mass

“…Epithelioid sarcoma is a very uncommon soft tissue tumour of mesenchymal origin 1 4 5 7. These tumours can be divided into distal or proximal types, occurring, respectively, at upper and lower extremities or in the trunk, abdomen or pelvis 1 4.…”

“…These tumours can be divided into distal or proximal types, occurring, respectively, at upper and lower extremities or in the trunk, abdomen or pelvis 1 4. The proximal type presents a more aggressive behaviour characterised by rapid expanding of the tumour, elevated local recurrence even after surgery with negative margins and propensity for early metastasis 4–8…”

“…Prior studies have shown that risk of recurrence is most closely related to inadequate resection of margins (<2 cm) and more often takes place within 6 months after surgery 1 2 4 7. Locoregional lymphadenectomy should not be performed by routine, but it is indicated when lymph nodes seem suspicious.…”

“…Poor prognosis is best correlated to histological high-grade tumours, lymph node involvement, vascular invasion, tumour sizes larger than 2 cm, deep location and presence of necrosis 1 4 6. Local recurrence is very common and distant metastases arise in 60% of cases 1 4 6 7.…”

“…Local recurrence is very common and distant metastases arise in 60% of cases 1 4 6 7. The 5-year overall survival rates for proximal type are worse than for those mentioned for distal type, which vary between 50% and 80% 4 7…”