Intrahepatic sarcomatous cholangiocarcinoma

A 77-year-old man, diagnosed with a liver tumor, was referred to our hospital. Abdominal ultrasonography demonstrated a low echoic mass in the liver S2 region, and abdominal CT confirmed the presence of a round low-density mass 7 cm in diameter. Enhanced angio-computed tomography (CT) showed a ring-like form with a pale periphery. In the delayed phase of angio-CT, the inside of the mass was enhanced, showing septal stricture. Abdominal magnetic resonance imaging (MRI) revealed a heterogenous low intensity area in T1-weighted images, with a clear high intensity border becoming apparent in T2-weighted images. Stretching of the hepatic artery was evident on the arterial phase of angiography, while an avascular area was apparent in the lateral segment of the liver in the portal phase. Lateral segmentectomy was performed. The size of the tumor was 6 x 6 x 5 cm. On macroscopic cross section, it was white and clearly demarcated from the surrounding tissue. Microscopic observation of H&E-stained specimens did not show any glandular formation. The tumor consisted of an irregular fascicular arrangement of spindle-shaped and round cells with poor intercellular adhesion. While there was no region containing differentiated epithelial components, silver impregnation staining revealed structures resembling regenerating bile ducts. The tumor cells were positive for wide-keratin, and for vimentin staining. Tumor cells were carcinoembryonic antigen (CEA)-positive and alpha-feto protein (AFP)-negative. From the above findings, the tumor was judged to have originated from epithelium rather than from mesenchymal elements. The final diagnosis was intrahepatic cholangiocarcinoma with secondary sarcomatous transformation, rather than hepatocellular carcinoma.