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

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); Herlyn-Werner-Wunderlich syndrome; diethylstilbestrol; genitourinary malformation; vaginal adenosis.

Figure 1

Schematic of the case. There was a fistula hole in the oblique septum, which did not cause severe menstrual blood retention but resulted in prolongation of menstruation.

Figure 2

Excision of vaginal lesions. The yellow arrows indicate the vaginal squamous epithelia, the blue arrows indicate the CCA lesions, the red arrows indicate the vaginal adenosis lesions, the green arrows indicate the atypical vaginal adenosis, and the dark arrow indicates the area of transformation from atypical vaginal adenosis to CCA (hematoxylin and eosin staining).