Elsevier

Gynecologic Oncology

Volume 126, Issue 3, September 2012, Pages 481-490
Gynecologic Oncology

Review
Clear cell carcinoma of the ovary: A review of the literature

https://doi.org/10.1016/j.ygyno.2012.04.021Get rights and content

Abstract

Objective

Different histologic types of epithelial ovarian cancer may represent different diseases with unique clinical and molecular characteristics. Clear cell carcinoma (CCC) of the ovary has been reported as having a worse prognosis than high grade serous epithelial ovarian cancer (EOC). This article critically reviews the literature pertinent to the pathology, pathogenesis, diagnosis, management, and outcome of patients with ovarian CCC.

Methods

MEDLINE was searched for all research articles published in English between January 01, 1977 and January 30, 2012 which reported on patients diagnosed with ovarian CCC. Given the rarity of this tumor, studies were not limited by design or number of reported patients.

Results

Ovarian CCC tumors represent 5–25% of ovarian cancers. Its histologic diagnosis can be challenging, resulting often times in misclassification of these tumors. Ovarian CCC tends to present at earlier stages and has been associated with endometriosis, ARID1A and PIK3CA mutations. When compared to stage-matched controls, patients with early-stage ovarian CCCs may have a better prognosis than patients with high-grade serous tumors. For those with advanced stage disease, high-grade serous histology confers a better prognosis than ovarian CCC. Patients with Stage IC–IV have a relatively poor prognosis and efforts should center in discovery of more effective treatment strategies.

Conclusions

Ovarian CCC is a biologically distinct entity, different from high-grade serous EOC. Future studies should explore the role of targeted therapies in the management of ovarian CCC.

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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