Long-term survival of intrahepatic cholangiocarcinoma with hilar lymph node metastasis and portal vein involvement

We report the case of a very rare 6-year disease-free survivor of intrahepatic cholangiocarcinoma with hilar lymph node metastasis and portal vein involvement. A 76-year-old female with liver dysfunction was referred to our institution. Contrast-enhanced computed tomography showed a 5-cm low-density tumor with irregular marginal enhancement in the left and caudate lobes of the liver. Cholangiography revealed complete obstruction of the left hepatic bile duct. Angiography showed obstruction of the left branch of the portal vein. Metastasis to the hilar lymph nodes was disclosed at surgery. The patient underwent left hepatectomy with caudate lobectomy, resection of the extrahepatic bile duct, and lymphadenectomy. The total vascular exclusion of the liver was used for hepatectomy and reconstruction of the portal vein. Microscopically, the tumor was a poorly differentiated adenocarcinoma with many infiltrating lymphocytes, and extensive necrosis was present within the tumor. The experience gained in the present case suggests that aggressive surgery may be a potential approach to provide a hope of long-term survival for patients with intrahepatic cholangiocarcinoma despite the presence of regional lymph node metastasis and vascular invasion.