Ovarian clear cell carcinoma (oCCC) is a distinctive subtype of ovarian carcinoma, with peculiar genetic and environmental risk factors, precursor lesions, molecular events during oncogenesis, patterns of spread, and response to treatment. Because of low response to chemotherapy and poor prognosis in advanced stages, there is growing interest in investigating the molecular pathways involved in oCCC development, in order to individualize novel/molecular targeted therapies. Until now, the main molecular genetic changes associated with oCCC remain to be identified, and, although several molecular changes have been reported in clear cell tumors, most studies have analyzed a limited number of cases; therefore, the true prevalence of those changes is not known. The present review will present the clinicopathologic features of oCCC, from morphology to molecular biology, discussing the diagnostic and treatment challenges of this intriguing ovarian carcinoma.
*Laboratory of Clinical Research and Advanced Diagnostics, IRCCS-CROB, Rionero in Vulture, PZ
†Pathology Unit, Cannizzaro Hospital, Catania
§Diagnostic Surgical Pathology Unit, Tor Vergata University
¶Department of Pathology, Catholic University of the Sacred Heart, Rome
∥Department of Oncology, University of Turin at San Luigi Hospital, Orbassano, Turin, Italy
‡Department of Pathology, Herlev and Gentofte Hospital, University of Copenhagen, Copenhagen, Denmark
F.F. and G.F.Z. contributed equally.
The authors declare no conflict of interest.
Reprints: Giuseppina Improta, PhD, IRCCS-CROB Via Padre Pio 1, 85028, Rionero in Vulture, PZ, Italy (e-mail: firstname.lastname@example.org).
Received January 15, 2018
Accepted February 28, 2018