Primary Ewing Sarcoma Presenting as a Vulvar Mass in an Adolescent: Case Report and Review of Literature.

BACKGROUND: Extraosseous Ewing sarcoma (ES) tumors presenting in the genitourinary tract are highly uncommon. Few cases of primary vulvar and vaginal cases of ES have been published. CASE: A 15-year-old adolescent presented with a bothersome 5-cm mass located on her left labium minorum. Following excision, a diagnosis of a primary ES was made. The patient was treated with multiagent chemotherapy and was doing well 20 months after treatment completion. SUMMARY AND CONCLUSION: Based on the few available case reports and our reported case, it appears that extraosseous ES arising in superficial sites such as the vulva have better prognosis and should be treated with complete excision and multiagent chemotherapy.