Herlyn-Werner-Wunderlich syndrome (HWWS)-associated gynecological malignancies: A case report and literature review.

Herlyn - Werner - Wunderlich 综合征 （ HWWS ） 相关妇科恶性肿瘤 1 例报告并文献复习.

• 发表时间: Oct 2022
• 来源期刊: Gynecol Oncol Rep
• DOI: 10.1016/j.gore.2022.101051
• PMID: 35923167
• 文章类型: Case Reports
• 作者列表: Mabuchi S,Hayashida H,Kubo C,Takemura M,Kamiura S,Mabuchi S,Hayashida H,Kubo C,Takemura M,Kamiura S

关键词: Cervical cancer; Clear cell carcinoma; Herlyn-Werner-Wunderlich syndrome; Cervical cancer; Clear cell carcinoma; Herlyn-Werner-Wunderlich syndrome

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。