Clear cell adenocarcinoma of the cervix in a child without in utero exposure to diethylstilbestrol:: A case report and review of the literature

We describe the case of a primary cervical tumor in a 6-year-old child that was originally suspected to be an embryonal rhabdomyosarcoma botryoides. Histologic analysis revealed a clear cell adenocarcinoma. Despite a direct search and questioning for maternal exposure to diethylstilbestrol, this was not documented. Clear cell adenocarcinoma is an extremely rare neoplasm that should be kept in the differential diagnosis of cervicovaginal lesions in children, even in the absence of a clinical history of in utero diethylstilbestrol exposure. We discuss the hypothesized pathogenesis and review the literature on this unusual tumor.