Virchows Archiv (1994) 425:445-448 © Springer-Verlag 1994 M. Michaela Ott - Monika Rehn • Justus G. Miiller Achim Gruss - Joachim Martius • Thomas Steck Hans K. Mtiller-Hermelink Vaginal clear cell carcinoma in a young patient with ectopic termination of the left ureter in the vagina Received: 17 June 1994 / Accepted: 24 August 1994 Abstract The association of clear cell adenocarcinoma of the vagina and vaginal adenosis with prenatal expo- sure to diethylstilbestrol (DES) is well-documented in the United States. In Europe, however, DES was never used in the therapy of threatened abortion and, therefore, clear cell adenocarcinoma and vaginal adenosis re- mained rare diseases. We report on the clinical and pathological features of a case of clear cell adenocarci- noma of the upper vagina in a 17-year-old German girl, who had a history of hypoplasia of the left kidney with an ectopic termination of the ureter in the upper vagina, removed surgically 2 years before. No previous report of a similar coincidence of vaginal clear cell carcinoma and a congenital disorder of the genitourinary tract exists. Congenital anomaly of the ureter interfering with the de- velopment and the differentiation of the distal MiJllerian tract and its epithelium might have provided a similar histological basis for carcinogenesis in our patient to that in those provided exposed to DES. Key words Clear cell adenocarcinoma • Vaginal Ureteral ectopia Introduction Clear cell carcinoma of the vagina is a rare tumour occur- ing in the upper vagina of children and young adults. In about two-thirds of all cases reported, a history of intra- uterine exposure to diethylstilbestrol (DES) was con- firmed in the United States (8), pointing to the possible role of DES in the pathogenesis of this rare neoplasm. In M. M. Ott ( ~ ) - J. G. Mfiller • H. K. Mt~ller-Hermelink Institute of Pathology, University of WiJrzburg, Josef-Schneider-Strasse 2, D-97080 Wtirzburg, Germany M. Rehn • J. Martius. T. Steck Department of Gynaecology and Obstetrics, University of Wtirzburg, Wtirzburg, Germany A. Gruss Department of Urology, University of Wi~rzburg, WiJrzburg, Germany Europe where DES had seldom been used in pregnant women, clear cell carcinoma of the vagina as well as vag- inal adenosis are extremely rare. While the hypothesis of DES-related oncogenesis in the USA was strengthened by the findings in animal models (10, 1 1), the histogene- sis of vaginal clear cell carcinoma without prenatal expo- sure to DES remains obscure, though there are no differ- ences regarding morphology nor the clinical aspects of these tumours. We report on a case of vaginal clear cell carcinoma in a patient without preceding exposure to DES, but with a history of a hypoplastic left kidney and ectopic termina- tion of the ureter in the upper vagina. Clinical history A 17-year-old nulliparous woman presented with conti- nous vaginal bleeding. She was diagnosed as ihaving an ectopic ostium of the left ureter into the left lateral fornix of the vagina at the age of 6. The ureter and a hypoplastic left kidney had been surgically removed in 1991 when the patient was 15. No vaginal tumour had been found and there was no history of intrauterine exposure to hormones by maternal intake of DES of treatment with agents known to be oestrogen modulators such as tamoxifen or danazol. On examination with the vaginoscope a polypoid mass was seen in the upper vagina and the right anterior wall. The cervix uteri could not be identified behind this tumour. Vaginal endosonography and CT scanning re- vealed a normal-sized uterus and ovaries, the tumour be- ing restricted to the vagina without evidence of intrauter- ine or abdominal spread. The right kidney and ureter were of normal size and shape on intravenous pyelo- gram. The patient was treated by radical hysterectomy, par- tial vaginectomy, and bilateral pelvic lymph node dissec- tion with preservation of the ovaries. Following surgery, the patient received intensive intravaginal radiotherapy (40 Gy, 5 fractions) using high dose brachytherapy.