Clear cell carcinoma of the ovary
Synonyms
Ovarian clear-cell carcinoma,Overview
Clear-cell carcinoma of the ovary (CCCO) is a distinct entity of epithelial ovarian cancer in terms of clinical, histopathological, or genetic features. The incidence of CCCO is different by ethnicity but the reason is not clear yet. Overall prognosis of CCCO is good because most CCCO is found in stage I. However, advanced disease is associated with a very poor prognosis and resistance to standard treatment. The same is true for recurrent disease. Therefore, genetic analysis of CCCO is important to find the right target(s) and better therapeutic approaches. Because of its rarity, international collaboration is necessary to conduct randomized clinical trials for CCCO.
Treatment
So far, treatment strategy for CCCO has been basically the same as for other histologic types of epithelial ovarian cancers. For early-stage CCCO, comprehensive surgical staging should be performed, including bilateral salpingo-oophorectomy, total hysterectomy, pelvic and periaortic lymphadenectomy, omentectomy, and peritoneal biopsies. Since CCCO is considered to be a high-risk group, adjuvant chemotherapy has been recommended even if the stage is IA. However, with the present knowledge on chemotherapy resistance in this disease, the usefulness of that approach seems questionableOn the other hand, the role of adjuvant radiotherapy for stage IC/II CCCO may be of interest to further study after the Canadian report that a subset of such patients might benefit from this approach.For advanced disease, an attempt to maximally debulk should be undertaken to achieve complete removal of all visible tumors. The importance of that was further illustrated by a large retrospective study by Takano et al. showing that complete surgery with no residual macroscopic disease was the only independent prognostic factor