A case of intrahepatic cholangiocarcinoma with the left portal vein tumor thrombus (Vp3. containing a sarcomatous component

A 70-year-old man complaining of epigastralgia visited our hospital for the treatment of a hepatic mass. The serology for viral hepatitis was negative, but his serum CA19-9 level was elevated. Contrast-enhanced computed tomography demonstrated a heterogeneously enhanced tumor, 6 cm in diameter, in the medial segment of the liver, the tumor thrombus extending to the left portal vein and the intrahepatic bile ducts dilatation of the left liver. Under a diagnosis of mass-forming type intrahepatic cholangiocarcinoma, extended left hepatectomy was performed. Pathologically, the tumor consisted of a moderately differentiated adenocarcinoma component, a solid proliferative component and a spindle-shaped sarcomatous component, and there was gradual transition among the different components. The portal vein tumor thrombus was composed of the spindle-shaped sarcomatous component. Eight months after surgery, CT showed multiple liver and mediastinal lymph node metastases. He then underwent systemic chemotherapy, and remains alive for 27 months after the initial surgery. Case reports of intrahepatic cholangiocarcinoma with portal vein tumor thrombus are rare, and this case is, to the best of our knowledge, the first report of intrahepatic cholangiocarcinoma with portal vein tumor thrombus containing a sarcomatous component. We present here the clinicopathological characteristics of this disease and review the previous reports.