Clear cell carcinoma of the ovary: Epidemiology, pathological and biological features, treatment options and clinical outcomes

• Published in: Gynecologic oncology Vol. 162; no. 3; pp. 741 - 750
• Main Authors: Gadducci, Angiolo, Multinu, Francesco, Cosio, Stefania, Carinelli, Silvestro, Ghioni, Mariacristina, Aletti, Giovanni Damiano
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.09.2021
• Subjects: Adenocarcinoma, Clear Cell - diagnosis; Adenocarcinoma, Clear Cell - epidemiology; Adenocarcinoma, Clear Cell - pathology; Adenocarcinoma, Clear Cell - therapy; Biological Factors - therapeutic use; Chemotherapy, Adjuvant - methods; Clear cell carcinoma of the ovary; Cytoreduction Surgical Procedures; Endometriosis - complications; Female; Hematology, Oncology, and Palliative Medicine; Humans; Molecular pathways; Neoplasm Staging; Obstetrics and Gynecology; Ovarian Neoplasms - diagnosis; Ovarian Neoplasms - epidemiology; Ovarian Neoplasms - pathology; Ovarian Neoplasms - therapy; Pathology; Prognosis; Salpingo-oophorectomy - methods; Surgery; Targeted therapy; Pathology; Molecular pathways; Prognosis; Targeted therapy; Surgery; Clear cell carcinoma of the ovary
• DOI: 10.1016/j.ygyno.2021.06.033
• ISSN: 0090-8258; 1095-6859

Clear cell carcinoma of the ovary is a rare and distinct histotype of epithelial ovarian carcinomas. Women diagnosed with clear cell carcinomas are usually younger and diagnosed at earlier stages than those with the most common high-grade serous histology. Endometriosis is considered a main risk factor for the development of clear cell carcinoma of the ovary, and it can be considered a precursor of of this tumor, as it is identified in more than 50% of patients with clear cell carcinoma. Different molecular pathways and alterations heve been identified in ovarian clear cell carcinoma, including the most common mutations of AT-rich interaction domain 1A [ARID1A] and phosphatidylinositol-4,5-bisphosphate 3-kinase [PIK3] catalytic subunit alpha [PIK3CA]. The prognosis of patients at early stage is favorable, while patients with advanced or recurrent disease experience a poor oncologic outcomes. Despite a lower rate of responses due to an intrinsic chemoresistance, the treatment strategy for advanced disease resembles the treatment of high-grade serous carcinoma, which includes aggressive cytoreductive surgery and platinum-based chemotherapy. For this reason, the role of adjuvant chemotherapy in patients with stage I disease undergoing complete surgical staging is still under debate. Alternative treatments, including biological agents that target different pathways constitute the most promising treatment strategies, and well-designed, collaborative international trials should be designed in order to improve the oncologic outcomes and the quality of life of patients with this aggressive disease. •CCC is a relatively uncommon carcinoma of the ovary, often associated with endometriosis•ARID1A mutations, and PIK3CA mutations are the most frequent gene alterations in CCC•Although the response to platinum-based chemo is not clearly defined, it remains the standard of the first-line therapy•Adjuvant chemotherapy in stage IA CCC is under debate•Targeted therapies represent a novel approach to specifically improve the outcome of patients with CCC