We report 2 patients with Herlyn-Werner-Wunderlich syndrome, 1 with advanced endometrioid adenocarcinoma of the semiobstructed side of the uterine cervix and 1 with primary clear cell carcinoma of the obstructed side of the upper vagina.
Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare Müllerian duct anomaly characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Although several cases of carcinoma in a uterus didelphys have been reported, no case reports of cervical or vaginal carcinoma in HWWS were found in the English literature. Herein we report the occurrence of endometrioid adenocarcinoma in the semiobstructed uterine cervix and clear cell carcinoma in the obstructed side of the vaginal wall in 2 patients with HWWS.
Case Reports
Case 1
A 33 year old woman, gravida 2, para 2 (2 cesarean sections), presented at our department with atypical genital bleeding. At the time of her first delivery, uterine didelphys, semiobstructed hemivagina with a canal diameter of approximately 1 cm, and right-sided renal and ureteral agenesis were noted. Furthermore, the patient had no history of in utero diethylstilbestrol (DES) exposure.
Initial pelvic examination revealed no abnormal findings in the visible part of the uterine cervix; however, macroscopic carcinoma was observed at the vaginal septum. Although cytology of the visible part of the uterine cervix showed normal findings, biopsy specimen from the vaginal septum showed endometrioid adenocarcinoma ( Figure 1 ). Moreover, human papillomavirus (HPV) was not detected by 2 different examination methods. Furthermore, magnetic resonance imaging (MRI) revealed a cervical tumor on the right side with urinary bladder invasion, uterine didelphys, and left renal agenesis ( Figure 2 ).
We diagnosed this case as International Federation of Gynecology and Obstetrics (FIGO) stage IVa primary uterine cervical or vaginal carcinoma in HWWS. The biopsy specimen showed viable adenocarcinoma cells, even after paclitaxel and carboplatin combination therapy, external radiation therapy. Therefore, we decided to perform anterior pelvic exenteration with vaginectomy for which we obtained fully informed consent. Surgically resected specimens showed carcinoma at the right side of the uterine cervix ( Figure 3 ).
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