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Primary vaginal clear cell adenocarcinoma accompanied by Herlyn-Werner-Wunderlich syndrome without prenatal diethylstilbestrol exposure: a case report
Authors:Ling Mei  Juan Zou  Qijun Chen  Wei Jiang  Yueyue Chen
Affiliation:1. Department of Gynecology, West China Second Hospital of Sichuan University, Chengdu 610041, Sichuan, China ; 2. Key Laboratory of Birth Defects and Related Diseases of Women and Children of Ministry of Education, West China Second Hospital of Sichuan University, Chengdu 610041, Sichuan, China ; 3. Department of Pathology, West China Second Hospital of Sichuan University, Chengdu 610041, Sichuan, China
Abstract:We report a rare case of a 40 years old woman with primary vaginal clear cell adenocarcinoma (CCA) accompanied by Herlyn-Werner-Wunderlich syndrome (HWWS) and without any prenatal diethylstilbestrol exposure. Pathological results indicated that the CCA was surrounded by adenosis and that there was a benign to malignant transformation of several glands in the epithelium, so the CCA was believed to have arisen from the adenosis. The literature review disclosed that genitourinary anomalies might be associated with an increased risk of adenocarcinoma, but the mechanism was not clear.
Keywords:Clear cell adenocarcinoma (CCA)   diethylstilbestrol   genitourinary malformation   Herlyn-Werner-Wunderlich syndrome   vaginal adenosis
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