Cervical cancer in a patient with uterus didelphys and obstructive hemivagina, ipsilateral renal anomaly (OHVIRA) syndrome

Abstract

We report a rare case of cervical adenocarcinoma diagnosed and treated in a woman with uterine didelphys, obstructive hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome. A 49-year-old multiparous woman applied to our institution with menorrhagia and intermenstrual bleeding since 6 months. On pelvic examination, a grossly normal cervix was noted. A 7-cm firm mass was palpated in the left pelvic region extending to the vaginal level. Also, a 4-cm fluctuant mass was palpated on the left vaginal wall. On pelvic ultrasound, a 40-mm cystic mass with internal echoes resembling an endometrioma was visualized adjacent to the left superior vaginal wall. Upper abdominal ultrasound revealed left unilateral renal dysplasia, which was further confirmed by magnetic resonance imaging (MRI) (Fig. 1). Laboratory, pap-smear and endocervical curettage (ECC) results were normal. Endometrial biopsy taken with a pipelle endometrial sampling cannula was reported as endometrioid adenocarcinoma. Laparotomy was performed and peritoneal washings were obtained for cytology. Upon exploration, uterine didelphys (UD) with normal adnexa on both sides were discovered (Fig. 2). After complete resection of the uterus with bilateral tubes and ovaries, a blind vaginal stump on the left side was visualized. A brown-colored fluid was drained from the left vaginal lumen. There was a small opening between the left and right vaginal lumens. The operation was completed with bilateral pelvic-paraaortic lymph node dissection (LND) and omentectomy. Final pathology was reported as UD with a 10 9 8 mm well-differentiated primary endometrioid adenocarcinoma of the right cervix. Endometrial tissues on both uteruses were free of any tumoral involvement. Retroperitoneal lymph nodes, omentum and peritoneal cytology were negative for any tumoral spread. The patient was informed about the pathology results. Further treatment options including surgical treatment or chemoradiation were discussed. The patient preferred surgical treatment. Radical parametrectomy and proximal vaginectomy were performed. Postoperative follow-up period was uneventful. On pathological examination, bilateral parametria and vaginal tissues were free of tumor. The patient was consulted to the radiation oncology department of an affiliated hospital. Regular follow-up without adjuvant treatment was recommended, and she was discharged from the hospital. Short-term (15 months) follow-up visits were normal without any treatment related complications or signs of recurrence. Obstructive hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome (Herlyn–Werner–Wunderlich syndrome) is a rare type of congenital Mullerian anomaly (CMA), which is frequently associated with UD [1, 2]. This syndrome results from lateral fusion defect of the Mullerian ducts with asymmetric vaginal obstruction. The classical urinary manifestation of OHVIRA syndrome is ipsilateral renal agenesis. However, as in our case, dysplastic or duplicated kidneys have also been reported [3]. Women with CMA are generally diagnosed in adolescence or reproductive period. Symptoms and clinical findings vary greatly, depending on the present anomaly. The woman presented here did not report any symptoms M. Kaba T. Gungor E. Baser (&) B. Ozdal Department of Gynecologic Oncology, Zekai Tahir Burak Women’s Health Education and Research Hospital, Talatpasa Bulvari, Samanpazari, Altindag, Ankara, Turkey e-mail: eralpbaser@gmail.com