Clear cell carcinoma of the ovary

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Clear cell carcinoma of the ovary
Diagnosis in short

Ovarian clear cell carcinoma. H&E stain.

Synonyms ovarian clear cell carcinoma, ovarian clear cell adenocarcinoma, clear cell adenocarcinoma of the ovary

LM cystic/tubular architecture (may be papillary and/or solid), clear cytoplasm (may be sparse), hobnail morphology - apical surface larger than basal surface ("nuclei bulge into the lumen"), +/-hyaline droplets, prominent nucleoli
LM DDx serous carcinoma of the ovary, other serous carcinomas, other clear cell tumours (e.g. clear cell renal cell carcinoma), Arias-Stella reaction within endometriosis
IHC HNF-1beta +ve, WT-1 -ve, CK7 +ve, CD10 -ve, CK20 -ve
Gross mass, yellow colour, usu. unilateral
Site ovary - see ovarian tumours

Associated Dx endometriosis, pulmonary embolism
Prevalence uncommon
Prognosis poor
Clin. DDx other ovarian tumours, other pelvic and abdominal tumours

Clear cell carcinoma of the ovary, also ovarian clear cell carcinoma (abbreviated OCCC), is an aggressive malignant tumour of the ovary.

It often forms glands; thus, it is also known as ovarian clear cell adenocarcinoma, and clear cell adenocarcinoma of the ovary.



  • Worse prognosis versus other surface epithelial tumours.[1]
    • Poor response to platinum-based chemotherapy.[2]
    • ~2.5x risk for thromboembolism compared to other ovarian carcinomas.[3]


  • Asians appear to be at an increased risk.[2]
  • Thought to be related to endometrioid carcinoma.[4]
  • Association with endometriosis:[2]
    • Increased risk of ovarian clear cell carcinoma ~ 3x.
    • Seen in 50-70% of ovarian CCC.





  • Cystic/tubular architecture - important low power feature.
    • May be papillary and/or solid.
  • Clear cells - cytoplasm is clear - key feature. †
  • Hobnail morphology - apical surface larger than basal surface.
    • "Nuclei bulge into the lumen".
  • Hyaline droplets -- common, as in clear cell renal cell carcinoma.
    • Eosinophilic bodies within lumen.
    • Seen in approximately 1/4 of cases.[2]
  • Nucleoli - prominent.


  • † Clear cell adenocarcinoma does not have to have clear cells. Yes, this is stupid; it is like papillary thyroid carcinoma -- which often isn't papillary -- see no truth in names.
    • The hobnail morphology is important if this is the case.





  • HNF-1beta +ve.[5][6]
    • Usu. -ve in serous carcinoma.
  • WT-1 -ve.
    • Usu. +ve in serous carcinoma.

Panel for high grade serous vs. clear cell:[7]

  • ER (+ve in serous),[8] HNF-1beta (+ve in clear cell), WT-1 (+ve in serous).


See also


  1. Hauptmann S, Köbel M (2005). "[Prognostic factors in ovarian carcinoma]" (in German). Verh Dtsch Ges Pathol 89: 92-100. PMID 18035678.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 Offman, SL.; Longacre, TA. (Sep 2012). "Clear cell carcinoma of the female genital tract (not everything is as clear as it seems).". Adv Anat Pathol 19 (5): 296-312. doi:10.1097/PAP.0b013e31826663b1. PMID 22885379.
  3. Duska, LR.; Garrett, L.; Henretta, M.; Ferriss, JS.; Lee, L.; Horowitz, N. (Mar 2010). "When 'never-events' occur despite adherence to clinical guidelines: the case of venous thromboembolism in clear cell cancer of the ovary compared with other epithelial histologic subtypes.". Gynecol Oncol 116 (3): 374-7. doi:10.1016/j.ygyno.2009.10.069. PMID 19922988.
  4. Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 1098. ISBN 0-7216-0187-1.
  5. Tsuchiya, A.; Sakamoto, M.; Yasuda, J.; Chuma, M.; Ohta, T.; Ohki, M.; Yasugi, T.; Taketani, Y. et al. (Dec 2003). "Expression profiling in ovarian clear cell carcinoma: identification of hepatocyte nuclear factor-1 beta as a molecular marker and a possible molecular target for therapy of ovarian clear cell carcinoma.". Am J Pathol 163 (6): 2503-12. PMID 14633622.
  6. Online 'Mendelian Inheritance in Man' (OMIM) 189907
  7. Köbel M, Kalloger SE, Carrick J, et al. (January 2009). "A limited panel of immunomarkers can reliably distinguish between clear cell and high-grade serous carcinoma of the ovary". Am. J. Surg. Pathol. 33 (1): 14–21. doi:10.1097/PAS.0b013e3181788546. PMID 18830127.
  8. DeLair, D.; Oliva, E.; Köbel, M.; Macias, A.; Gilks, CB.; Soslow, RA. (Jan 2011). "Morphologic spectrum of immunohistochemically characterized clear cell carcinoma of the ovary: a study of 155 cases.". Am J Surg Pathol 35 (1): 36-44. doi:10.1097/PAS.0b013e3181ff400e. PMID 21164285.