Primary vaginal clear cell adenocarcinoma accompanied by Herlyn-Werner-Wunderlich syndrome without prenatal diethylstilbestrol exposure: a case report

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.