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Detailed overview on rare malignant ovarian tumors. 2020
Stefanie Aust, and
Lauriane Eberst, and
Olivier Tredan, and
Christine Rousset-Jablonski, and
Isabelle Treilleux, and
Pierre Méeus, and
Nicolas Chopin, and
Fred Beurrier, and
Amandine Charreton, and
Daval Véronique, and
Amina Hallouz, and
Agnès Coulon, and
Alexis Ricoeur, and
Charles Mastier, and
Amine Bouhamama, and
Séverine Racadot, and
Mojgan Devouassoux-Shisheboran, and
Véronique Haddad, and
Isabelle Ray-Coquard
Comprehensive Cancer Center, Department of Obstetrics and Gynecology, Division of General Gynecology and Gynecologic Oncology, Medical University of Vienna, Vienna, Austria.
The group of rare malignant ovarian tumors includes the group of germ cell tumors, sex cords stromal ovarian tumors, small cell carcinoma, malignant Brenner tumors, rare epithelial tumors such as mucinous carcinoma, clear cell carcinoma, or low-grade serous carcinoma, as well as ovarian carcinosarcoma. Together they comprise about 10% of all ovarian tumors. Due to their low prevalence and their heterogeneity, data and treatment recommendations are limited. Even though all ovarian tumors are staged according to the FIGO staging of epithelial ovarian tumors, treatment differs especially in germ cell tumors and sex cords 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. As can be expected already due to their divergent precursor lesions, these malignancies are substantially different but united by their rarity. This overview article gives a comprehensive summary on the pathology and clinical presentation, as well as therapy recommendations of a selection of those rare ovarian tumors, based on the latest national guidelines and related important publications.
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