Detailed overview on rare malignant ovarian tumors
Bulletin du cancer · Mar 2020 · narrative review
This is a narrative review summarizing pathology, clinical presentation, and treatment recommendations for a selection of rare malignant ovarian tumors. The authors state these tumors are heterogeneous, comprise about 10% of ovarian tumors, and that data and treatment recommendations are limited; they note that staging follows FIGO but treatment differs for germ cell and sex cord-stromal tumors. The review is based on recent national guidelines and related important publications.
Reported effect: Proportion of ovarian tumors 10%
Key findings
- Rare malignant ovarian tumors covered include germ cell tumors, sex cord-stromal ovarian tumors, small cell carcinoma, malignant Brenner tumors, and rare epithelial tumors such as mucinous, clear cell and low-grade serous carcinomas, as well as ovarian carcinosarcoma.
- Together these rare malignant ovarian tumors comprise about 10% of all ovarian tumors.
- Because of low prevalence and heterogeneity, data and treatment recommendations for these tumors are limited.
- Although all ovarian tumors are staged according to FIGO staging of epithelial ovarian tumors, treatment differs especially for germ cell tumors and sex cord-stromal ovarian tumors.
- Non-epithelial ovarian tumors can arise from a variety of ovarian precursor cells such as germ cells, granulosa cells, theca cells, or stromal fibroblasts, reflecting divergent origins.
- The article provides a comprehensive summary of pathology, clinical presentation, and therapy recommendations for a selection of rare ovarian tumors based on the latest national guidelines and important publications.
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed