Intrahepatic Cholangiocarcinoma

Aim : The authors give a complete overview on this disease from epidemiology to treatment. Background : Cholangiocarcinoma (CCA) is an epithelial tumor with features of cholangio- cyte differentiation. Most patients suffer from a nonresectable disease since presentation and the exitus occurs within 12 months from diagnosis. Biliary epithelial carcinogenesis is a multistep process that involves the transition from hyperplasia to dysplasia to carcinoma. The clinical approach should be multidisciplinary, and the diagnosis should be considered when there is a histological finding of adenocarcinoma without any other evi dences of an extrahepatic primitive neoplasia. Surgical resection with histologically nega- tive margins is the only curative treatment. Nevertheless for unresectable patients, there are several other approaches: systemic chemotherapy is the widely used treatment, but a large proportion of patients could be suitable for liver-directed therapies. These options include transarterial chemoembolization (TACE), radioembolization (TARE), hepatic arte - rial infusion (HAI), percutaneous ablation, and external beam radiation therapy (EBRT). Conclusion : Intrahepatic cholangiocarcinoma is a relatively rare disease with a poor prog- nosis. Diagnosis is based on imaging, but pathological anatomy plays an important role. Surgery is still the gold standard treatment; nevertheless, unresectable patients could be treated in a multimodality strategy with a significant improvement in terms of survival .