Vaginal clear cell adenocarcinoma with associated Müllerian duct anomalies, renal agenesis and situs inversus: Report of a case with no known in-utero exposure with Diethyl stilboestrol

A 27 year old female presented with two months history of continuous vaginal bleeding. On evaluation, she was diagnosed as a case of vaginal clear cell adenocarcinoma (VCCA) along with associated Müllerian duct anomalies including didelphys uterus with double vagina; left sided renal agenesis and situs inversus. Although VCCA is strongly linked to in-utero exposure with Diethyl stillboestrol (DES), this patient had no known history regarding such exposure. Also, only a few cases have been described in the literature in which this tumour was seen in association with Müllerian duct anomalies and renal agenesis; in the absence of known DES exposure.