Hepatoid carcinoma of the ovary: a literature review

Synopsis Hepatoid carcinoma of the ovary (HCO) exhibits morphological and immunophenotypic features highly similar to those of hepatocellular carcinoma. Immunohistochemical staining for alpha-fetoprotein, hepatocyte paraffin antigen 1, and glypican-3 is an important auxiliary approach for the clinical diagnosis of HCO. Currently, most treatment regimens for this disease are developed with reference to clinical management protocols for high-grade epithelial ovarian cancer. Hepatoid carcinoma of the ovary (HCO) is an extremely rare, distinct histological subtype of primary ovarian adenocarcinoma that exhibits remarkable clinicopathological similarity to hepatocellular carcinoma (HCC). The histogenetic origin of HCO remains controversial, with hypotheses proposing derivation from ovarian surface epithelium or extragonadal non-germ cell precursors. This neoplasm primarily affects postmenopausal women, typically presenting as unilateral or bilateral cystic-solid ovarian masses accompanied by significantly elevated serum alpha-fetoprotein (AFP) levels. HCO is characterized by high biological aggressiveness, with most cases diagnosed at an advanced stage leading to a uniformly poor prognosis. Owing to its extreme rarity, existing research on HCO is largely limited to sporadic case reports. This systematic review aggregates eligible HCO cases published from 1987 to January 2026, conducts pooled analysis and comprehensive synthesis of clinical characteristics, pathological features, treatment strategies and molecular findings, with the aim of providing evidence-based references for clinical diagnosis and therapeutic decision-making, and establishing a theoretical foundation for future translational research.