Clear cell carcinomas of the uterine corpus and cervix are rare gynecological cancers with limited information regarding the pathogenesis and biology. At present, the approach to management is the same as for patients with the more common histological subtypes of endometrioid endometrial cancer and adenocarcinoma of the cervix. Surgical resection is the standard treatment for patients with early-stage disease, but there is no evidence-based approach to direct the management of patients with more advanced-stage disease at presentation or with recurrent disease. We review the epidemiology, pathology, and what is known about both uterine corpus and cervical clear cell cancers and make management recommendations.
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