ABSTRACT
BACKGROUND: Combined hepatocellular-cholangiocarcinoma is a rare primary hepatic tumour, showing both hepatocellular as well as biliary epithelium differentiation. Its diagnosis is often delayed, as it occurs in young patients without comorbidities and with non-specific symptoms. Most cases are confused with other types of cancer, especially fibrolamellar liver cancer, which is more frequent and has similar clinical and radiological features. CLINICAL CASE: The case is presented of a 26 year old woman with a giant combined hepatocellular-cholangiocarcinoma with difficulties in its diagnosis and a complicated surgical approach. DISCUSSION: The definitive diagnosis of this disease is defined by the histological demonstration of cholangiolar and hepatocellular differentiation, with surgical treatment always being the best choice, but with lower survival than classic hepatocellular carcinoma and cholangiocarcinoma. In some patients with unfavourable prognostic factors, adjuvant chemotherapy mainly directed cholangiolar component can be given. CONCLUSION: The current incidence of combined hepatocellular-cholangiocarcinoma varies from 2 to 5% of cases, and is one of the rarest histological types in the world. The large size and hypervascularisation of the tumour makes a surgical approach difficult in these patients, while the rare histological features require a more detailed study of the piece and the application of immunohistochemical techniques to confirm the diagnosis.