Clear cell adenocarcinoma of the vagina in a young female, treated by combination chemotherapy (local and systemic chemotherapy), complicated with chromosomal abnormality.

Although cancer of the vagina, a rare form of genital cancer, is usually squamous cell carcinoma in women over the age of 50 years, vaginal clear cell adenocarcinomas (CCA) are exceedingly rare. The pathogenesis of clear cell adenocarcinoma is uncertain, but as a clinical entity, it is most commonly associated with diethylstilbestrol exposure in utero. We report a rare case of primary vaginal CCA in a 17-year-old female who was not exposed to diethylstilbesterol in utero. The case, which was complicated with chromosomal abnormality, was treated with combination chemotherapy. To our knowledge, this is the first reported case of vaginal CCA in a woman with chromosomal abnormality and bicornate uterus.