Epithelioid sarcoma of the vulva. Evidence suggesting a more aggressive behavior than extra-genital epithelioid sarcoma.

The authors describe two cases of epithelioid sarcoma of the vulva and summarize the clinical and pathologic experience with this vulvar neoplasm. Findings indicate that epithelioid sarcoma of the vulva usually occurs in the labia majora of young women, but may occur in later life. Clinically it may mimic a Bartholin's duct cyst, thus leading to inadequate treatment. Pathologic findings are characterized by sheets and nests of acidophilic, polygonal cells. Follow-up study suggests that vulvar epithelioid sarcoma behaves more aggressively than extragenital epithelioid sarcoma. Four of five patients died of metastatic sarcoma. The courses of three patients were quite rapid, whereas a fourth patient had multiple recurrences over a long period, which is typical of extragenital epithelioid sarcoma. Complete initial excision is important. All patients with local recurrence died with distant metastases. Vascular invasion also indicates a poor outcome.