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Clear cell carcinoma of the ovary: Epidemiology, pathological and biological features, treatment options and clinical outcomes.
Gadducci, Angiolo; Multinu, Francesco; Cosio, Stefania; Carinelli, Silvestro; Ghioni, Mariacristina; Aletti, Giovanni Damiano.
Affiliation
  • Gadducci A; Department of Clinical and Experimental Medicine, Division of Gynecology and Obstetrics, University of Pisa, Pisa, Italy.
  • Multinu F; Department of Gynecologic Surgery, IRCCS European Institute of Oncology, Milan, Italy.
  • Cosio S; Department of Clinical and Experimental Medicine, Division of Gynecology and Obstetrics, University of Pisa, Pisa, Italy.
  • Carinelli S; Division of Pathology and Laboratory Medicine, IRCCS European Institute of Oncology, Milan, Italy.
  • Ghioni M; Division of Pathology and Laboratory Medicine, IRCCS European Institute of Oncology, Milan, Italy.
  • Aletti GD; Department of Gynecologic Surgery, IRCCS European Institute of Oncology, Milan, Italy; Department of Oncology and Hemato-Oncology, University of Milan, Milan, Italy. Electronic address: giovanni.aletti@ieo.it.
Gynecol Oncol ; 162(3): 741-750, 2021 09.
Article in En | MEDLINE | ID: mdl-34247767
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.
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Ovarian Neoplasms / Adenocarcinoma, Clear Cell Type of study: Diagnostic_studies / Prognostic_studies / Risk_factors_studies / Screening_studies Aspects: Patient_preference Limits: Female / Humans Language: En Journal: Gynecol Oncol Year: 2021 Document type: Article Affiliation country: Italy Country of publication: United States

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Ovarian Neoplasms / Adenocarcinoma, Clear Cell Type of study: Diagnostic_studies / Prognostic_studies / Risk_factors_studies / Screening_studies Aspects: Patient_preference Limits: Female / Humans Language: En Journal: Gynecol Oncol Year: 2021 Document type: Article Affiliation country: Italy Country of publication: United States