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Abstract
Introduction/Background Rare malignancies, with an annual incidence of less than 6/100,000, pose challenges in understanding their natural history and prognostic features due to their scarcity. Diverse therapeutic approaches are required due to significant patient variability in age, histological subtypes, and stage. The World Health Organization classifies rare ovarian cancers into three subtypes: epithelial tumors, non-epithelial tumors, and germ cell tumors, each with distinct subgroups impacting prognosis and treatment.
Methodology In this retrospective observational study conducted from 2017 to 2021 including 56 patients, we aimed to investigate the epidemiological profile, diagnostic approaches, and therapeutic strategies employed in the management of rare ovarian cancer. We also focused on the staging procedures performed within the abdominal radiology section and sought to establish correlations with the anatomopathological results obtained in our Centre.
Results Our results reveal that rare ovarian tumors across a broad age range (16–74 years, average 50 years), with menopausal women representing 53% and 47.9% being multiparous. Common discovery circumstances include increased abdominal volume (54%) and chronic pelvic pain (31.8%) with a median consultation delay of 7 months. Ultrasound and CT scans were key diagnostics. Histologically, rare epithelial tumors prevailed (e.g., Mucinous carcinoma 22.8%). Non-epithelial tumors, such as Sex cord stromal tumors (19.2%), and Germ cell tumors (8.77%), were frequent. Regarding staging, Ovarian neoplasms were surgically staged using the FIGO system, with 60% radiological-pathological correlation, mainly through CT scans. Radical surgery occurred in 46%, and 25% underwent neo-adjuvant chemotherapy. Monitoring relied on tumor markers and CT scans. Study end results: 70% complete remission, 12% progression despite chemotherapy, and unfortunate demise of 2 patients.
Conclusion Rare ovarian cancers encompass diverse tumors with varying clinical presentations and natural histories. Multidisciplinary tumor boards at national and international levels aim to consolidate expertise, yet significant challenges, particularly regarding surgical approaches and access to innovative therapies, persist, requiring ongoing collaboration.
Disclosures The authors declare no conflicts of interest and report that no grants or financial support from any organization were received in connection with this study.
