Cholangioblastic variant of intrahepatic cholangiocarcinoma is a rare, extremely malignant liver tumor. Currently, only six clinical cases have been described worldwide. Following the bibliographic research, the authors of the paper introduced main features of the tumor, methods of its diagnosis and treatment. A clinical observation of a patient with cholangioblastic variant of intrahepatic cholangiocarcinoma is presented. The course of the disease was complicated by tumor thrombosis of the left hepatic and inferior vena cava with thrombus flotation in the right atrium. The paper describes the stages of the disease diagnosis and surgical treatment of the patient, providing insight into the patient surveillance in such cases. Given the low incidence of such neoplasms, each clinical case reported in the scientific literature is of great value and practical interest.
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