A case of vaginal clear cell adenocarcinoma complicated with congenital anomalies of the genitourinary tract and metanephric remnant without prenatal diethylstilbestrol exposure

Vaginal clear cell adenocarcinoma (CCA) is well known to be associated with prenatal diethylstilbestrol exposure. We present a vaginal CCA with congenital anomalies of the genitourinary tract without prenatal diethylstilbestrol exposure. A 54-year-old woman complained of a 3-month history of genital bleeding. The examination revealed CCA at the anterior vagina and congenital anomalies. An anterior pelvic exenteration was performed. Macroscopically, bicornuate uterus, vaginal septum and left ureteral agenesis were found. Microscopically, vaginal CCA coexisted with adenosis and both metanephric and mesonephric remnants. The vaginal CCA was supposed to derive from coexisting adenosis. The adenosis was also supposed to occur as a congenital basis, together with genitourinary tract anomalies. Relations between congenital anomalies of the genitourinary tract and vaginal adenocarcinoma were suspected, resultantly.