Abstract:
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.
摘要:
Herlyn-Werner-Wunderlich综合征(HWWS)是一种罕见的先天性泌尿生殖系统异常,其特征是子宫双子宫,单侧盲半阴道,和同侧肾发育不全。我们介绍了一个非常罕见的HWWS相关宫颈癌病例,其中直到患者经历绝经后阴道出血才发现生殖器异常。一名74岁的日本未产妇女出现阴道出血。治疗前检查显示子宫双子宫,阻塞的半阴道/半宫颈,肾发育不全,和癌症发展的残余阻塞的半阴道/半宫颈。患者被诊断为HWWS和HWWS相关的阴道或宫颈癌,接受根治性手术治疗,并在组织病理学上证实了宫颈透明细胞癌(CCC)的诊断。文献综述显示,患有HWWS的女性中CCC的发病率增加。