Tanase Yasuhito, Yoshida Hiroshi, Naka Tomoaki, Kitamura Shoichi, Natsume Takashi, Kobayashi Kato Mayumi, Uno Masaya, Ishikawa Mitsuya, Ali Mahmoud Ali Ahmed, Kato Tomoyasu
Department of Gynecology, National Cancer Central Hospital, 5-1-1, Tsukiji, Chuo-ku, Tokyo 104-0045, Japan.
Department of Diagnostic Pathology, National Cancer Central Hospital, 5-1-1, Tsukiji, Chuo-ku, Tokyo 104-0045, Japan.
World J Oncol. 2021 Feb;12(1):34-38. doi: 10.14740/wjon1362. Epub 2021 Feb 24.
Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare Mullerian duct anomaly characterized by an obstructed hemivagina, ipsilateral renal agenesis, and uterine didelphys. There are only a few published case reports of OHVIRA syndrome, and cases of OHVIRA syndrome associated with cancer have rarely been reported. In fact, there is only one published report of a case with clear cell carcinoma (CCC) of the cervix. Here, we report a case of CCC of the cervix with OHVIRA syndrome that underwent abdominal radical hysterectomy; we also provide a short literature review of this topic. A 52-year-old woman presented with abnormal vaginal bleeding for 1 month 2 years after menopause. A pelvic examination and preoperative imaging showed uterine didelphys, an obstructed hemivagina with a mass measuring approximately 2 cm located in her left cervix, and an absence of her left kidney. A colposcopy biopsy reported CCC of the cervix. Clinical staging classified her with stage IB1 disease. Abdominal radical hysterectomy was performed. Her left ectopic ureter led to the left cervix and opened in the endometrium, resulting in a so-called ectopic ureter. Macroscopic examination of the excised specimens showed two cervixes, two corpora of the uterus, and a tumor measuring 1.0 × 2.0 cm on the left cervix. In addition to typical OHVIRA symptoms including uterine didelphys, obstructed hemivagina, and renal agenesis, several anatomical variants were present. The current case included those variants as well as an atrophic kidney with an ectopic ureter to the obstructed hemivagina. Based on the results of our case, clinicians should be aware of the risks of cancer and anatomical variants associated with OHVIRA syndrome.
梗阻性半阴道并同侧肾缺如（OHVIRA）综合征是一种罕见的苗勒管异常，其特征为梗阻性半阴道、同侧肾缺如和双子宫。关于OHVIRA综合征的已发表病例报告很少，与癌症相关的OHVIRA综合征病例更是鲜有报道。事实上，仅有一篇关于宫颈透明细胞癌（CCC）病例的已发表报告。在此，我们报告一例患有OHVIRA综合征的宫颈CCC患者，该患者接受了腹式根治性子宫切除术；我们还提供了关于该主题的简短文献综述。一名52岁女性在绝经后2年出现阴道异常出血1个月。盆腔检查和术前影像学检查显示为双子宫、左侧宫颈有一约2 cm大小肿块的梗阻性半阴道以及左肾缺如。阴道镜活检报告为宫颈CCC。临床分期为IB1期疾病。实施了腹式根治性子宫切除术。她的左侧异位输尿管通向左侧宫颈并开口于子宫内膜，形成所谓的异位输尿管。切除标本的宏观检查显示有两个宫颈、两个子宫体以及左侧宫颈上一个1.0×2.0 cm大小的肿瘤。除了包括双子宫、梗阻性半阴道和肾缺如等典型的OHVIRA症状外，还存在几种解剖变异。本例除了这些变异外，还包括一个萎缩肾以及一条通向梗阻性半阴道的异位输尿管。基于我们的病例结果，临床医生应意识到与OHVIRA综合征相关的癌症风险和解剖变异。
