Abstract

Hepatoid carcinoma of the ovary is an ultra-rare and highly aggressive epithelial malignancy, sharing prominent resemblance in both morphology and immunophenotype with hepatocellular carcinoma. Due to the rarity of the tumour and its histologic overlap with metastatic hepatic tumours and other ovarian neoplasms, HCO is often misdiagnosed, which delays the initiation of appropriate treatment, leading to poor clinical outcomes. The goal of this narrative review was to summarise the main clinicopathological features, immunohistochemical profile, molecular alterations, and current approaches to the management of hepatoid ovarian carcinoma, with an emphasis on key diagnostic pitfalls and emerging therapeutic perspectives. A Focused narrative review of published English-language case reports, case series, and literature reviews was performed using major biomedical databases, covering from the initial description of HCO in 1987 to recent reports. Relevant articles were analysed to extract data on patient demographics, clinical presentation, imaging characteristics, histopathology, immunohistochemical markers, serum biomarkers, molecular findings, treatment strategies, and clinical outcomes. Less than 50 well-documented cases of HCO have been hitherto reported, mainly affecting perimenopausal and postmenopausal women. The majority of patients present with large adnexal masses, strikingly increased serum alpha-fetoprotein levels, and advanced-stage disease at diagnosis. Histologically, tumours demonstrate hepatoid differentiation, characterized by the consistent expression of AFP, HepPar-1, glypican-3, and SALL4. Recent reports, including those utilizing next-generation sequencing, have revealed recurrent TP53 alterations, as well as additional potentially actionable molecular pathways. Despite multimodal treatment, recurrence rates are high, and overall survival remains limited. Hepatoid carcinoma of the ovary requires heightened diagnostic vigilance, standardised immunohistochemical evaluation, and increased incorporation of molecular profiling in order to improve diagnostic precision and enable individualised therapeutic strategies in this exceptionally rare malignancy.