Mabuchi Seiji, Hayashida Harue, Kubo Chiaki, Takemura Masahiko, Kamiura Shoji
Department of Gynecology, Osaka International Cancer Institute, 3-1-69, Otemae, Chuo-ku, Osaka-shi, Osaka 541-8567, Japan.
Department of Diagnostic Pathology, Osaka International Cancer Institute, 3-1-69, Otemae, Chuo-ku, Osaka-shi, Osaka 541-8567, Japan.
Gynecol Oncol Rep. 2022 Jul 26;43:101051. doi: 10.1016/j.gore.2022.101051. eCollection 2022 Oct.
Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital urogenital anomaly characterized by uterine didelphys, unilateral blind hemivagina, and ipsilateral renal agenesis. We present a very rare case of HWWS-associated cervical cancer in which the presence of a genital anomaly was not noticed until the patient experienced postmenopausal vaginal bleeding. A 74-year-old nulliparous Japanese woman presented with vaginal bleeding. Pre-treatment workup revealed uterine didelphys, obstructed hemivagina/hemicervix, renal agenesis, and cancer development from the remnant-obstructed hemivagina/hemicervix. The patient was diagnosed with HWWS and HWWS-associated vaginal or cervical cancer, treated with radical surgery, and a diagnosis of clear cell carcinoma (CCC) of the uterine cervix was histopathologically confirmed. A literature review revealed an increased incidence of CCC in women with HWWS.
赫林-韦纳-温德利希综合征（HWWS）是一种罕见的先天性泌尿生殖系统异常，其特征为双子宫、单侧盲端半阴道和同侧肾缺如。我们报告一例极为罕见的HWWS相关宫颈癌病例，该患者直到绝经后出现阴道出血才被发现存在生殖器官异常。一名74岁未生育的日本女性出现阴道出血。治疗前检查发现双子宫、梗阻性半阴道/半宫颈、肾缺如以及残余梗阻性半阴道/半宫颈发生癌变。该患者被诊断为HWWS及HWWS相关阴道或宫颈癌，接受了根治性手术治疗，组织病理学证实为宫颈透明细胞癌（CCC）。文献综述显示，HWWS女性患者中CCC的发病率有所增加。
