Vulvar epithelioid hemangioendothelioma.

BACKGROUND

Epithelioid hemangioendothelioma (EH) is a rare vascular tumor whose localization in the vulva is extremely rare.

CASE: The present case report refers to a 19-year-old Brazilian woman admitted to hospital with an extensive lesion of granulomatous appearance on the right labium majus. Following confirmation of the diagnosis of epithelioid hemangioendothelioma, the only treatment consisted of hemivulvectomy and right inguinal lymphadenectomy. Patient was lost to follow-up for 8 years after recovery from surgery. She reappeared 4 years ago and is now being followed-up. There is no sign of recurrence of the disease.

CONCLUSION

EH is a lesion of unpredictable malignancy whose localization in the vulva is extremely rare. The first therapeutic option is surgical resection.