Primary vaginal adenocarcinoma unrelated to in utero exposure to diethylsti
lbestrol (DES) is very uncommon. We report a case of 65-year-old Japanese w
oman who presented with primary adenocarcinoma in the anterior wall of the
vagina, where the left ureter-like metanephric duct remnant abnormally term
inated. Histological examination in serial sections revealed the direct con
nection between the carcinoma and the metanephric duct remnant. Moreover, t
he remnant epithelium showed varying degrees of dysplastic changes, includi
ng carcinoma in situ in close proximity to the carcinoma. This patient also
had a bicornate uterus and left renal aplasia. To our knowledge, this is t
he first reported case of a primary vaginal adenocarcinoma arising from the
metanephric duct remnant. Although the precise mechanism involved in carci
nogenesis in this clinicopathological setting remains unknown, adenocarcino
ma should be included in the differential diagnosis of vaginal tumors in pa
tients with renal aplasia and/or an ectopic termination of the ureter or me
tanephric duct remnant, especially when the tumor is in the anterior wall.