Controversies in the management of mucinous ovarian tumors.

Abstract

Mucinous ovarian carcinoma (MOC) is a rare subtype of epithelial ovarian cancer, comprising less than 5% of all cases, and distinguished by its unique molecular, histologic, and clinical features. Its rarity and marked differences from other ovarian cancer subtypes have led to significant controversies regarding its diagnosis, surgical strategies, and systemic therapies. Accurate differentiation between primary and metastatic MOCs remains a critical challenge because of their overlapping features with gastrointestinal cancers, often leading to misclassification. This can result in suboptimal management and impaired patient outcomes, thus highlighting the importance of high-quality pathologic reviews. The surgical approach to MOC is highly controversial. In early-stage disease, fertility-sparing surgery should be systematically considered in young patients, although its feasibility requires careful consideration. In addition, systematic staging lymphadenectomy, which has been de-escalated for patients with expansile MOC, is recommended for those with early-stage infiltrative MOC. In advanced-stage disease, where tumors are often bulky and chemoresistant, the benefits of extensive cytoreduction must be balanced against surgical morbidities. MOC poses significant challenges for systemic treatment owing to its poor response rate to standard ovarian cancer chemotherapy regimens. Alternative therapeutic strategies offer promise but lack robust clinical validation, including gastrointestinal-based regimens, HER2-targeted antibody-drug conjugates (eg, trastuzumab deruxtecan), and immune checkpoint inhibitors for microsatellite unstable MOC. Furthermore, pre-clinical and early phase trials have suggested the potential of combination strategies, including RAS pathway and WEE1 inhibitors. Addressing these controversies requires a multidisciplinary approach that underscores the importance of histologic subtyping and molecular profiling to guide personalized treatment. International collaboration is essential for overcoming the rarity of MOC by enabling larger studies and global registries. These efforts are pivotal for improving diagnostic accuracy, expanding therapeutic options, and, ultimately, enhancing outcomes in patients with this challenging malignancy.