RESUMO
The clear cell adenocarcinoma of uterine cervix is very rare tumor, and only 4-9% of entire adenocarcinoma appears to be diagnosed as clear cell adenocarcinoma. Its risk factor and pathogenesis are not exactly known, but intrauterine exposure to DES (diethyl stilbestrol) and associated non-steroidal estrogen during pregnancy before 18weeks is one known risk factor, and also hormonal change or genetic cause are suggested as the risk factors. The peak age of its occurrence has bimodal pattern, which are groups before 24 years-old and after 45 years-old, and clear cell adenocarcinoma arising in latter group is not associated with intrauterine DES exposure. It is also reported that 25% of young women who has clear cell adenocarcinoma had no history of hormonal exposure. The treatment and prognosis is similar to other kinds of uterine cervical adenocarcinoma. With a short literature review, we are reporting a case of 6-year-old girl who visited our clinic because of vaginal mass naturally fallen off with a minor bleeding which was finally diagnosed as clear cell adenocarcinoma, and had no maternal history of DES exposure during pregnancy.