Localized Neurofibromatosis of the Female Genitourinary Tract

Abstract

The internal female genital tract is rarely involved in neurofibromatosis. This report details a case of diffuse neurofibroma with prominent Wagner-Meissner body differentiation involving uterine cervix and endocervix, vagina, and vulva. The urinary bladder, urethra, and one ureter were also extensively affected. The anatomic localization of the lesions in this case is consistent with segmental neurofibromatosis restricted to the distribution of the second, third, and fourth sacral nerves. Failure to consider the possibility of genitourinary involvement in neurofibromatosis, the potential for localized manifestations in the absence of other easily recognized stigmata, and the subtle histologic features in small tissue biopsies may cause difficulty in diagnosis.