The occurrence of endometrial adenocarcinoma in a patient with basal cell nevus syndrome

Basal cell nevus syndrome is rare multisystem disorder. Its genital implications in female patients is thought to be limited to ovarian fibroma. This article describes endometrial adenocarcinoma in association with basal cell nevus syndrome in a 37-year-old female with multiple basal cell carcinomas, mandibular and maxillary bone cysts, right ovarian fibroma, and scoliosis. Histopathologic examination of her endometrial biopsy, performed for irregular uterine bleeding, revealed endometrial hyperplasia. Her bleeding failed to respond to progestin therapy, and a repeat endometrial biopsy revealed a well differentiated endometrial adenocarcinoma. Health-care providers are encouraged to refer these patients to gynecologists. Regular gynecologic examination as well as appropriate evaluation of abnormal menstrual bleeding should be emphasized.