Article ID Journal Published Year Pages File Type
3943109 Gynecologic Oncology 2011 9 Pages PDF
Abstract

ObjectivesRecent literature has highlighted histological types of ovarian carcinoma as distinct diseases, each with unique clinical and molecular features. Historically, the diagnosis of ovarian clear cell carcinoma (CCC) has been of concern to both patients and physicians due to reports that CCC is associated with a worse prognosis than the more common serous type of ovarian carcinoma (HGSC). This review discusses the unique features of ovarian CCC.MethodsIn June of 2010, a group of researchers and clinicians convened in Vancouver to review and discuss the clinical, pathological, molecular, and treatment-related features of CCC.ResultsCCC is the second most common type of ovarian epithelial cancer, representing 5–25% of ovarian carcinomas. It is characterised by its association with endometriosis, and frequent mutations of ARID1A and PIK3CA. Low-stage CCC appears to have a better outcome than stage matched HGSC, while the opposite is true for high-stage disease, suggesting that the current standard treatments applied to HGSC are ineffective for CCC.ConclusionsOvarian CCC is highly distinct from HGSC, and a clearer understanding of the basic biology of this disease is needed. Alternative therapies should be explored: irradiation and targeting disease-specific molecular markers should be examined in greater detail. Finally, novel approaches to clinical trial design are needed due to the smaller numbers of patients affected.

Research highlights►Ovarian clear cell carcinoma is distinct from other ovarian cancers. ►It is associated with endometriosis, and mutations of ARID1A and PIK3CA. ►Novel models for trials and study should be explored

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