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22-08-2017 | Ovarian cancer | Article

Contemporary Treatment Strategies for Rare Epithelial Ovarian Cancers

Current Obstetrics and Gynecology Reports

Authors: Jennifer E. Bergstrom, Amanda N. Fader, David M. Gershenson

Publisher: Springer US


Purpose of Review

Epithelial ovarian carcinoma consists of not one but five distinct histologic subtypes which include high-grade and low-grade serous carcinomas, clear cell, endometrioid, and mucinous carcinoma. Until recently, women with all histologic subtypes have been “grouped together” in cooperative group trials and treated in a similar fashion.

Recent Findings

Recent advances have demonstrated that each subtype differs in molecular and genetic differentiation, pathogenesis, clinical behavior, and response to treatments. Since the establishment of the Gynecologic Oncology Group (GOG) Rare Tumor Committee in 2005, there has been increasing emphasis placed upon novel treatment strategies for these low incidence malignancies. There has been mounting evidence to suggest that a “one-size-fits-all” treatment approach is not appropriate—especially for low-grade serous, clear cell, endometrioid, and mucinous carcinomas as they all tend to be relatively chemoresistant to typical platinum/taxane-based chemotherapy regimens utilized for high-grade serous carcinomas.


We present a review of the rare epithelial ovarian cancer subtypes, including current best clinical practices for the management of each tumor type.

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