Aust Stefanie, Eberst Lauriane, Tredan Olivier, Rousset-Jablonski Christine, Treilleux Isabelle, Méeus Pierre, Chopin Nicolas, Beurrier Fred, Charreton Amandine, Véronique Daval, Hallouz Amina, Coulon Agnès, Ricoeur Alexis, Mastier Charles, Bouhamama Amine, Racadot Séverine, Devouassoux-Shisheboran Mojgan, Haddad Véronique, Ray-Coquard Isabelle
Comprehensive Cancer Center, Department of Obstetrics and Gynecology, Division of General Gynecology and Gynecologic Oncology, Medical University of Vienna, Vienna, Austria.
Department of Medical Oncology, centre Léon-Bérard, Lyon, France.
Bull Cancer. 2020 Mar;107(3):385-390. doi: 10.1016/j.bulcan.2020.01.011. Epub 2020 Feb 27.
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.
罕见的恶性卵巢肿瘤包括生殖细胞肿瘤、性索间质卵巢肿瘤、小细胞癌、恶性勃勒纳瘤、罕见的上皮性肿瘤（如黏液性癌、透明细胞癌或低级别浆液性癌）以及卵巢癌肉瘤。它们总共约占所有卵巢肿瘤的10%。由于其发病率低且具有异质性，相关数据和治疗建议有限。尽管所有卵巢肿瘤都按照上皮性卵巢肿瘤的FIGO分期进行分期，但治疗方法在生殖细胞肿瘤和性索间质卵巢肿瘤中尤其不同。非上皮性卵巢肿瘤可源自多种卵巢前体细胞，如生殖细胞、颗粒细胞、卵泡膜细胞或基质成纤维细胞。正如因其不同的前体病变所预期的那样，这些恶性肿瘤有很大差异，但因其罕见性而被归为一类。这篇综述文章基于最新的国内指南和相关重要出版物，对部分罕见卵巢肿瘤的病理学、临床表现以及治疗建议进行了全面总结。
