Article Text

Download PDFPDF
Gynecologic Cancer InterGroup (GCIG) Consensus Review for Clear Cell Carcinoma of the Ovary
  1. Aikou Okamoto, MD, PhD*,
  2. Rosalind M. Glasspool, MBBS, PhD, FRCP,
  3. Seiji Mabuchi, MD, PhD,
  4. Noriomi Matsumura, MD, PhD§,
  5. Hiroyuki Nomura, MD, PhD,
  6. Hiroaki Itamochi, MD, PhD,
  7. Masashi Takano, MD, PhD#,
  8. Tadao Takano, MD, PhD**,
  9. Nobuyuki Susumu, MD, PhD,
  10. Daisuke Aoki, MD, PhD,
  11. Ikuo Konishi, MD, PhD§,
  12. Alan Covens, MD, LMCC, FRCSC,,
  13. Jonathan Ledermann, MD, FRCP,,
  14. Delia Mezzazanica, PhD§§,
  15. Christopher Steer, MBBS, FRACP∥∥,
  16. David Millan, BSc, MB, ChB, FRCPath¶¶,
  17. Iain A. McNeish, MD, PhD##,
  18. Jacobus Pfisterer, MD, PhD***,
  19. Sokbom Kang, MD, PhD††,,
  20. Laurence Gladieff, MD,,,
  21. Jane Bryce, MSN§§§ and
  22. Amit Oza, MD, FRPCPC, MBBs∥∥∥
  1. *Jikei University School of Medicine, Tokyo, Japan;
  2. Beatson West of Scotland Cancer Centre, Glasgow, United Kingdom;
  3. Osaka University Graduate School of Medicine, Osaka, Japan;
  4. §Kyoto University Graduate School of Medicine, Kyoto, Japan;
  5. Keio University, Tokyo, Japan;
  6. Tottori University School of Medicine, Tottori, Japan;
  7. #National Defense Medical College, Saitama, Japan;
  8. **Clinical Research, Innovation, and Education Center, Tohoku University Hospital, Sendai, Japan;
  9. ††Sunnybrook Hospital, Toronto, Canada;
  10. ‡‡UCL Cancer Institute, London, United Kingdom;
  11. §§Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy;
  12. ∥∥Border Medical Oncology, Victoria, Australia;
  13. ¶¶Department of Pathology, Glasgow Royal Infirmary, Glasgow, United Kingdom;
  14. ##Institute of Cancer Sciences, University of Glasgow Wolfson Wohl Cancer Research Centre, Beatson Institute for Cancer Research, Glasgow, United Kingdom;
  15. ***Gynecologic Oncology Center, Kiel, Germany;
  16. †††National Cancer Center, Goyang, Korea;
  17. ‡‡‡Institut Claudius Regaud, Toulouse, France;
  18. §§§National Cancer Institute, Naples, Italy; and
  19. ∥∥∥Princess Margaret Hospital, Toronto, Canada.
  1. Address correspondence and reprint requests to Aikou Okamoto, MD, PhD, Department of Obstetrics and Gynecology, The Jikei University School of Medicine, 3-25-8 Nishi-Shinbashi, Minato-ku Tokyo 105-8461 Japan. E-mail: aikou7000{at}


Abstract Clear cell carcinoma of the ovary (CCC) is a histologic subtype of epithelial ovarian cancer with a distinct clinical behavior. There are marked geographic differences in the prevalence of CCC. The CCC is more likely to be detected at an early stage than high-grade serous cancers, and when confined within the ovary, the prognosis is good. However, advanced disease is associated with a very poor prognosis and resistance to standard treatment. Cytoreductive surgery should be performed for patients with stage II, III, or IV disease. An international phase III study to compare irinotecan/cisplatin and paclitaxel/carboplatin as adjuvant chemotherapy for stage IIV CCC has completed enrollment (GCIG/JGOG3017). Considering the frequent PIK3CA mutation in CCC, dual inhibitors targeting PI3K, AKT in the mTOR pathway, are promising. Performing these trials and generating the evidence will require considerable international collaboration.

  • Clear cell carcinoma of the ovary (CCC)
  • Deep venous thrombosis
  • Glycogen
  • Hepatocyte nuclear factor-1ß
  • WT1
  • Pulmonary embolism
  • Ethnics
  • ARID1A
  • PIK3CA
  • PPM1D
  • PPP2R1A
  • KRAS
  • Cytoreductive surgery
  • Paclitaxel
  • Platinum
  • Irinotecan hydrochloride
  • Cisplatin

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.


  • The authors declare no conflicts of interest.