ReviewClear cell carcinoma of the ovary: A review of the literature
Highlights
► Ovarian CCC tumors tend to present at earlier stages. ► Ovarian CCC has been associated with endometriosis, ARID1A and PIK3CA mutations. ► Future studies should explore the role of targeted therapies in the management of ovarian CCC.
Introduction
Epithelial ovarian cancer is the most lethal gynecologic malignancy. Effective screening strategies are lacking and most women are diagnosed with advanced stage disease. An estimated 22,280 new cases of ovarian cancer will be diagnosed in the United States in 2012, with close to 15,500 deaths [1]. Epithelial ovarian cancer (EOC) accounts for 90–95% of all cases, while sex-cord stromal tumors and malignant germ cell tumors remain rare. Various randomized, controlled clinical trials have been carried out and their results largely guide the management of most women with EOC. These trials are not discussed in the present review.
Section snippets
Methods
This article reviews the English language literature for studies on clear cell ovarian cancer. A 35-year period MEDLINE (PubMed) search of English literature published between January 01, 1977 and January 30, 2012 was performed. All publications with the keyword “ovary” were combined and then searched for the keyword “clear cell.” Additional publications were identified via systematic review of all reference lists within publications retrieved from the MEDLINE search. Given the rarity of this
Conclusions
Ovarian CCC continues to be managed using the treatment paradigm that has been adopted for other EOCs. CCC represents a distinct clinical challenge with a unique and different biology when compared to high grade serous EOC. Given our present understanding of its unique biology, future investigations should focus on the identification of clinical, pathologic, and molecular features of select patients, such as those with Stage I disease, who may not necessitate adjuvant systemic therapy and may
Conflict of interest statement
None of the authors have any conflict of interests to report.
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