Definition
Clear cell cystadenoma and adenofibroma: Benign epithelial neoplasm composed of simple small to medium-sized glands lined by bland clear or oxyphilic cells with accompanying fibromatous stroma.
Clear cell borderline tumor: An epithelial neoplasm composed of crowded glands and/or cysts lined by clear or oxyphilic cells with low-grade nuclear atypia set within fibromatous stroma, without stromal invasion.
Clear cell carcinoma: A malignant epithelial neoplasm showing tubulocystic, papillary, and/or solid architecture, composed of clear (or oxyphilic) cuboidal and hobnail cells.
Clinical Features
Incidence
Benign clear cell tumors are extremely rare, with only small case series described in the literature (Zhao et al. 2011). Pure clear cell borderline tumors are rare, comprising <1% of all ovarian borderline tumors (Uzan et al. 2012). They are more commonly seen accompanying a clear cell carcinoma than as the exclusive element in a tumor. Clear cell carcinoma accounts for...
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References and Further Reading
Bennett, J. A., Dong, F., Young, R. H., & Oliva, E. (2015). Clear cell carcinoma of the ovary: Evaluation of prognostic parameters based on a clinicopathological analysis of 100 cases. Histopathology, 66(6), 808–815. https://doi.org/10.1111/his.12514.
Chandler, R. L., Damrauer, J. S., Raab, J. R., Schisler, J. C., Wilkerson, M. D., Didion, J. P., Starmer, J., Serber, D., Yee, D., Xiong, J., Darr, D. B., Pardo-Manuel de Villena, F., Kim, W. Y., & Magnuson, T. (2015). Coexistent ARID1A-PIK3CA mutations promote ovarian clear-cell tumorigenesis through pro-tumorigenic inflammatory cytokine signalling. Nature Communications, 6, 6118. https://doi.org/10.1038/ncomms7118.
Lim, D., Ip, P. P., Cheung, A. N., Kiyokawa, T., & Oliva, E. (2015). Immunohistochemical comparison of ovarian and uterine Endometrioid carcinoma, Endometrioid carcinoma with clear cell change, and clear cell carcinoma. The American Journal of Surgical Pathology, 39(8), 1061–1069. https://doi.org/10.1097/PAS.0000000000000436.
Machida, H., Matsuo, K., Yamagami, W., Ebina, Y., Kobayashi, Y., Tabata, T., Kanauchi, M., Nagase, S., Enomoto, T., & Mikami, M. (2019). Trends and characteristics of epithelial ovarian cancer in Japan between 2002 and 2015: A JSGO-JSOG joint study. Gynecologic Oncology, 153(3), 589–596. https://doi.org/10.1016/j.ygyno.2019.03.243.
Parra-Herran, C., Bassiouny, D., Lerner-Ellis, J., Olkhov-Mitsel, E., Ismiil, N., Hogen, L., Vicus, D., & Nofech-Mozes, S. (2019). p53, mismatch repair protein, and POLE abnormalities in ovarian clear cell carcinoma: An outcome-based Clinicopathologic analysis. The American Journal of Surgical Pathology, 43(12), 1591–1599. https://doi.org/10.1097/PAS.0000000000001328.
Peres, L. C., Cushing-Haugen, K. L., Kobel, M., Harris, H. R., Berchuck, A., Rossing, M. A., Schildkraut, J. M., & Doherty, J. A. (2019). Invasive epithelial ovarian cancer survival by Histotype and disease stage. Journal of the National Cancer Institute, 111(1), 60–68. https://doi.org/10.1093/jnci/djy071.
Uzan, C., Dufeu-Lefebvre, M., Fauvet, R., Gouy, S., Duvillard, P., Darai, E., & Morice, P. (2012). Management and prognosis of clear cell borderline ovarian tumor. International Journal of Gynecological Cancer, 22(6), 993–999. https://doi.org/10.1097/IGC.0b013e3182534acf.
Wiegand, K. C., Shah, S. P., Al-Agha, O. M., Zhao, Y., Tse, K., Zeng, T., Senz, J., McConechy, M. K., Anglesio, M. S., Kalloger, S. E., Yang, W., Heravi-Moussavi, A., Giuliany, R., Chow, C., Fee, J., Zayed, A., Prentice, L., Melnyk, N., Turashvili, G., … Huntsman, D. G. (2010). ARID1A mutations in endometriosis-associated ovarian carcinomas. The New England Journal of Medicine, 363(16), 1532–1543. https://doi.org/10.1056/NEJMoa1008433.
Zhao, C., Wu, L. S., & Barner, R. (2011). Pathogenesis of ovarian clear cell adenofibroma, atypical proliferative (borderline) tumor, and carcinoma: Clinicopathologic features of tumors with endometriosis or adenofibromatous components support two related pathways of tumor development. Journal of Cancer, 2, 94–106. https://doi.org/10.7150/jca.2.94.
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Tchrakian, N. (2023). Clear Cell Tumors, Pathology of the Ovary. In: Stolnicu, S., Ali-Fehmi, R. (eds) Gynecologic Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-97378-0_5656
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DOI: https://doi.org/10.1007/978-3-030-97378-0_5656
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