Skip to main content

WHO Classification of Tumors of the Ovary, Pathology of the Ovary

  • Reference work entry
  • First Online:
Gynecologic Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 11 Accesses

The World Health Organization (WHO) classification of female genital tumors was revised in the fifth edition published in 2020 (Table 1) (WHO 2020). Epithelial tumors remain subdivided by cell type into the following categories: serous, mucinous, endometrioid, clear cell, seromucinous, Brenner, and other. The classification of ovarian carcinoma is largely unchanged from the previous edition (Kurman et al. 2014), in which it was emphasized that the five main ovarian carcinoma histotypes (high-grade serous, low-grade serous, endometrioid, clear cell, and mucinous) are completely different diseases. The differences between these encompass risk factors, precursors, genomic changes and immunophenotype, patterns of disease spread, treatment responses, and outcomes. The category of mixed carcinoma, which had been excluded from the fourth edition, has been reintroduced in the fifth edition. Mixed ovarian carcinomas have been shown to be extremely rare (<1% of all ovarian neoplasms) and...

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 379.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 379.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References and Further Reading

  • de Kock, L., Terzic, T., McCluggage, W. G., et al. (2017). DICER1 mutations are consistently present in moderately and poorly differentiated Sertoli-Leydig cell Tumors. The American Journal of Surgical Pathology, 41, 1178–1187.

    Article  PubMed  Google Scholar 

  • Foulkes, W. D., Gore, M., & McCluggage, W. G. (2016). Rare non-epithelial ovarian neoplasms: Pathology, genetics and treatment. Gynecologic Oncology, 142, 190–198.

    Article  CAS  PubMed  Google Scholar 

  • Irving, J. A., Lee, C. H., Yip, S., et al. (2015). Microcystic stromal tumor: A distinctive ovarian sex cord-stromal neoplasm characterized by FOXL2, SF-1, WT-1, cyclin D1, and beta-catenin nuclear expression and CTNNB1 mutations. The American Journal of Surgical Pathology, 39, 1420–1426.

    Article  PubMed  Google Scholar 

  • Jelinic, P., Mueller, J. J., Olvera, N., et al. (2014). Recurrent SMARCA4 mutations in small cell carcinoma of the ovary. Nature Genetics, 46, 424–426.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Karnezis, A. N., Wang, Y., Ramos, P., et al. (2016). Dual loss of the SWI/SNF complex ATPases SMARCA4/BRG1 and SMARCA2/BRM is highly sensitive and specific for small cell carcinoma of the ovary, hypercalcaemic type. The Journal of Pathology, 238, 389–400.

    Article  CAS  PubMed  Google Scholar 

  • Karnezis, A. N., Wang, Y., Keul, J., et al. (2019). DICER1 and FOXL2 mutation status correlates with Clinicopathologic features in ovarian Sertoli-Leydig cell Tumors. The American Journal of Surgical Pathology, 43, 628–638.

    Article  PubMed  Google Scholar 

  • Kurman, R. J., Carcangiu, M. L., Herrington, C. S., & Young, R. H. (Eds.). (2014). WHO classification of tumours of female reproductive organs. Lyon: International Agency for Research on Cancer.

    Google Scholar 

  • Mackenzie, R., Talhouk, A., Eshragh, S., et al. (2015). Morphologic and molecular characteristics of mixed epithelial ovarian cancers. The American Journal of Surgical Pathology, 39, 1548–1557.

    Article  PubMed  PubMed Central  Google Scholar 

  • McCluggage, W. G., Irving, J. A., Chong, A. S., et al. (2018). Ovarian microcystic stromal Tumors are characterized by alterations in the Beta-catenin-APC pathway and may be an Extracolonic manifestation of familial adenomatous polyposis. The American Journal of Surgical Pathology, 42, 137–139.

    Article  PubMed  Google Scholar 

  • Pors, J., Segura, S., Chiu, D. S., et al. (2021). Clinicopathologic characteristics of mesonephric adenocarcinomas and mesonephric-like adenocarcinomas in the Gynecologic tract: A multi-institutional study. The American Journal of Surgical Pathology, 45, 498–506.

    Article  PubMed  PubMed Central  Google Scholar 

  • Rambau, P. F., McIntyre, J. B., Taylor, J., et al. (2017). Morphologic reproducibility, genotyping, and Immunohistochemical profiling do not support a category of Seromucinous carcinoma of the ovary. The American Journal of Surgical Pathology, 41, 685–695.

    Article  PubMed  Google Scholar 

  • Ramos, P., Karnezis, A. N., Craig, D. W., et al. (2014). Small cell carcinoma of the ovary, hypercalcemic type, displays frequent inactivating germline and somatic mutations in SMARCA4. Nature Genetics, 46, 427–429.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Shah, S. P., Köbel, M., Senz, J., et al. (2009). Mutation of FOXL2 in granulosa-cell tumors of the ovary. The New England Journal of Medicine, 360, 2719–2729.

    Article  CAS  PubMed  Google Scholar 

  • Silva, E. G., Deavers, M. T., Bodurka, D. C., & Malpica, A. (2006). Association of low-grade endometrioid carcinoma of the uterus and ovary with undifferentiated carcinoma: A new type of dedifferentiated carcinoma? International Journal of Gynecological Pathology, 25, 525–528.

    Article  Google Scholar 

  • Taylor, J., & McCluggage, W. G. (2015). Ovarian seromucinous carcinoma: Report of a series of a newly categorized and uncommon neoplasm. The American Journal of Surgical Pathology, 39, 983–992.

    Article  PubMed  Google Scholar 

  • Tischkowitz, M., Huang, S., Banerjee, S., et al. (2020). Small-cell carcinoma of the ovary, Hypercalcemic type-genetics, new treatment targets, and current management guidelines. Clinical Cancer Research, 26, 3908–3917.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Vroobel, K. M., & McCluggage, W. G. (2021). Ovarian clear cell Tumors associated with Seromucinous borderline tumor: A case series. International Journal of Gynecological Pathology. https://doi.org/10.1097/PGP.0000000000000768. Online ahead of print.

  • WHO Classification of Tumours Editorial Board. Female genital tumours [Internet]. Lyon (France): International Agency for Research on Cancer; 2020 [cited 2021 October 22]. (WHO classification of tumours series, 5th ed.; vol. 4). Available fro https://tumourclassification.iarc.who.int/chapters/34.

  • Witkowski, L., Carrot-Zhang, J., Albrecht, S., et al. (2014). Germline and somatic SMARCA4 mutations characterize small cell carcinoma of the ovary, hypercalcemic type. Nature Genetics, 46, 438–443.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Naveena Singh .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2023 Springer Nature Switzerland AG

About this entry

Check for updates. Verify currency and authenticity via CrossMark

Cite this entry

Singh, N. (2023). WHO Classification of Tumors of the Ovary, 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_5651

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-97378-0_5651

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-97377-3

  • Online ISBN: 978-3-030-97378-0

  • eBook Packages: MedicineReference Module Medicine

Publish with us

Policies and ethics