Cholangiolocellular Carcinoma: An Innocent-Looking Malignant Liver Tumor Mimicking Ductular Reaction

ABSTRACT

The authors present an interesting case of a 60-year-old man who underwent right hepatectomy for a diagnosis of hepatocellular carcinoma (HCC) on a background of noncirrhotic chronic hepatitis C. Pathologic examination confirmed the presence of HCC near the porta hepatis, which invaded the right portal vein branch. In addition, a well-demarcated 13.5 × 7.8 × 4.0 cm yellow and firm area upstream of the HCC was noted. This yellow area corresponded to a tumoral ductular proliferation, which cytologically was extremely bland, but invaded portal tracts and the adjacent liver parenchyma. This tumoral proliferation mimicked ductular reaction, except that it had more anastomosing structures and was associated with abundant hyalinized fibrotic stroma. Cytologically, the tumor cells had round to oval nuclei with fine chromatin, indistinct nucleoli, and scant cytoplasm. They exhibited immunohistochemical features of hepatic progenitor cells, i.e., expressing CK7, CK19, and N-CAM; and their malignancy was supported by the p53 and Ki67 immunoreactivity. The authors concluded that the patient had cholangiolocellular carcinoma with an aggressive hepatocellular carcinoma component. Cholangiolocellular carcinoma has been reported to be associated with chronic hepatitis C viral infection and to derive from hepatic progenitor cells, which explains why hepatocellular carcinoma and/or cholangiocarcinoma component may be present.

KEYWORDS

Cholangiolocellular carcinoma - hepatocellular carcinoma - hepatic progenitor cells

REFERENCES

• 1 Shiota K, Taguchi J, Nakashima O, Nakashima M, Kojiro M. Clinicopathologic study on cholangiolocellular carcinoma.  Oncol Rep. 2001;  8 (2) 263-268
• 2 Steiner P E, Higginson J. Cholangiolocellular carcinoma of the liver.  Cancer. 1959;  12 (4) 753-759
• 3 Nakanuma Y, Sripa B, Vatanasapt V et al.. Intrahepatic cholangiocarcinoma. In: Hamilton S R, Aaltonen L A, eds. WHO Classification of Tumors, Pathology and Genetics of Tumours of the Digestive System. Lyon: IARC Press; 2000: 173-180
• 4 Komuta M, Spee B, Vander Borght S et al.. Clinicopathological study on cholangiolocellular carcinoma suggesting hepatic progenitor cell origin.  Hepatology. 2008;  47 (5) 1544-1556
• 5 Kanamoto M, Yoshizumi T, Ikegami T et al.. Cholangiolocellular carcinoma containing hepatocellular carcinoma and cholangiocellular carcinoma, extremely rare tumor of the liver: a case report.  J Med Invest. 2008;  55 (1–2) 161-165
• 6 Fukukura Y, Hamanoue M, Fujiyoshi F et al.. Cholangiolocellular carcinoma of the liver: CT and MR findings.  J Comput Assist Tomogr. 2000;  24 (5) 809-812
• 7 Asayama Y, Tajima T, Okamoto D et al.. Imaging of cholangiolocellular carcinoma of the liver.  Eur J Radiol. 2010;  75 (1) e120-e125
• 8 Motosugi U, Ichikawa T, Nakajima H et al.. Cholangiolocellular carcinoma of the liver: imaging findings.  J Comput Assist Tomogr. 2009;  33 (5) 682-688
• 9 Theise N D, Saxena R, Portmann B C et al.. The canals of Hering and hepatic stem cells in humans.  Hepatology. 1999;  30 (6) 1425-1433
• 10 Gütgemann I, Haas S, Berg J P, Zhou H, Büttner R, Fischer H P. CD56 expression aids in the differential diagnosis of cholangiocarcinomas and benign cholangiocellular lesions.  Virchows Arch. 2006;  448 (4) 407-411
• 11 Kozaka K, Sasaki M, Fujii T et al.. A subgroup of intrahepatic cholangiocarcinoma with an infiltrating replacement growth pattern and a resemblance to reactive proliferating bile ductules: ‘bile ductular carcinoma’.  Histopathology. 2007;  51 (3) 390-400
• 12 Nakanuma Y, Sasaki M, Ikeda H et al.. Pathology of peripheral intrahepatic cholangiocarcinoma with reference to tumorigenesis.  Hepatol Res. 2008;  38 (4) 325-334
• 13 Matsuda M, Hara M, Suzuki T, Kono H, Fujii H. Synchronously resected double primary hepatic cancers - hepatocellular carcinoma and cholangiolocellular carcinoma.  J Hepatobiliary Pancreat Surg. 2006;  13 (6) 571-576
• 14 Durnez A, Verslype C, Nevens F et al.. The clinicopathological and prognostic relevance of cytokeratin 7 and 19 expression in hepatocellular carcinoma. A possible progenitor cell origin.  Histopathology. 2006;  49 (2) 138-151