Endodermal sinus tumor of the vulva in an infant

An endodermal sinus tumor arising in the vulva of a 22-month-old infant is reported and the features of 3 previously recorded endodermal sinus tumors of the vulva are reviewed. Despite radical surgery, radiation therapy, and chemotherapy the tumor was fatal within 6 months. Only one of the 3 previously described patients with this type of vulvar tumor has survived more than 2 years. That tumor was the smallest reported, being the only one under 2 cm in diameter.