Clear cell carcinoma of the ovary: Is there a role of histology-specific treatment?

Abstract. several clinical trials to establish standard treatment modality for ovarian cancers included a high abundance of patients with serous histologic tumors,which were quite sensitive to platinum-based chemotherapy. on the other hand,ovarian tumor with rare histologic subtypes such as clear cell or mucinous tumors have been recognized to show chemo-resistant phenotype,leading to poorer prognosis. especially,clear cell carcinoma of the ovary (ccc) is a distinctive tumor,deriving from endometriosis or clear cell adenofibroma,and response rate to platinum-based therapy is extremely low. it was implied that complete surgical staging enabled us to distinguish a high risk group of recurrence in ccc patients whose disease was confined to the ovary (pt1m0); however,complete surgical staging procedures could not lead to improved survival. moreover,the status of peritoneal cytology was recognized as an independent prognostic factor in early-staged ccc patients,even after complete surgical staging. in advanced cases with ccc,the patients with no residual tumor had significantly better survival than those with the tumor less than 1cm or those with tumor diameter more than 1cm. therefore,the importance of achieving no macroscopic residual disease at primary surgery is so important compared with other histologic subtypes. on the other hand,many studies have shown that conventional platinum-based chemotherapy regimens yielded a poorer prognosis in patients with ccc than in patients with serous subtypes. the response rate by paclitaxel plus carboplatin (tc) was slightly higher,ranging from 22% to 56%,which was not satisfactory enough. another regimen for ccc tumors is now being explored: irinotecan plus cisplatin,and molecular targeting agents. in this review article,we discuss the surgical issues for early-staged and advanced ccc including possibility of fertility-sparing surgery,and the chemotherapy for ccc disease. © 2012 takano et al.; licensee biomed central ltd.