Ma. Khalifa et al., THE OCCURRENCE OF ENDOMETRIAL ADENOCARCINOMA IN A PATIENT WITH BASAL-CELL NEVUS SYNDROME, Journal of the National Medical Association, 89(8), 1997, pp. 549-552
Basal cell nevus syndrome is a rare multisystem disorder. Its genital
implications in female patients is thought to be limited to ovarian fi
broma. This article describes endometrial adenocarcinoma in associatio
n with basal cell nevus syndrome in a 37-year-old Female with multiple
basal cell carcinomas, mandibular and maxillary bone cysts, right ova
rian fibroma, and scoliosis. Histopathologic examination of her endome
trial biopsy, performed for irregular uterine bleeding, revealed endom
etrial hyperplasia. Her bleeding failed to respond to progestin therap
y, and a repeat endometrial biopsy revealed a well differentiated endo
metrial adenocarcinoma. Health-care providers are encouraged to refer
these patients to gynecologists. Regular gynecologic examination as we
ll as appropriate evaluation of abnormal menstrual bleeding should be
emphasized.