Clinicopathological, radiologic, and molecular study of 23 combined hepatocellular-cholangiocarcinomas with stem cell features, cholangiolocellular type

• Published in: Human pathology Vol. 64; pp. 118 - 127
• Main Authors: Chen, Jun, He, Jian, Deng, Min, Wu, Hong-Yan, Shi, Jiong, Mao, Liang, Sun, Qi, Tang, Min, Fan, Xiang-Shan, Qiu, Yu-Dong, Huang, Qin
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.06.2017; Elsevier Limited
• Subjects: Adult; Aged; Aged, 80 and over; Bile; Bile Duct Neoplasms - diagnosis; Bile Duct Neoplasms - diagnostic imaging; Bile Duct Neoplasms - genetics; Bile Duct Neoplasms - pathology; Bile ducts; Biomarkers, Tumor - genetics; Biopsy; Carcinoma, Hepatocellular - diagnosis; Carcinoma, Hepatocellular - diagnostic imaging; Carcinoma, Hepatocellular - genetics; Carcinoma, Hepatocellular - pathology; Cell Proliferation; Cholangiocarcinoma; Cholangiocarcinoma - diagnosis; Cholangiocarcinoma - diagnostic imaging; Cholangiocarcinoma - genetics; Cholangiocarcinoma - pathology; Cholangiolocellular carcinoma; Classification; Dehydrogenases; Digestive system; DNA Mutational Analysis; Female; Hepatectomy; Hepatic tumors; Hepatitis; Hepatology; Humans; Immunohistochemistry; Isocitrate dehydrogenase; Isocitrate Dehydrogenase - genetics; Kaplan-Meier Estimate; Laboratories; Liver cancer; Liver Neoplasms - diagnosis; Liver Neoplasms - diagnostic imaging; Liver Neoplasms - genetics; Liver Neoplasms - pathology; Lymphatic system; Male; Medical prognosis; Medical records; Middle Aged; Molecular Diagnostic Techniques; Mutation; Neoplasm Invasiveness; Neoplasms, Complex and Mixed - diagnosis; Neoplasms, Complex and Mixed - diagnostic imaging; Neoplasms, Complex and Mixed - genetics; Neoplasms, Complex and Mixed - pathology; Neoplastic Stem Cells - pathology; Pathology; Patients; Predictive Value of Tests; Proportional Hazards Models; Stem cells; Studies; Surgery; Time Factors; Tomography, X-Ray Computed; Treatment Outcome; Tumor Burden; Tumorigenesis; Tumors; Viral infections; Hepatic tumors; Cholangiolocellular carcinoma; Isocitrate dehydrogenase; Bile ducts; Cholangiocarcinoma
• ISSN: 0046-8177; 1532-8392; 1532-8392
• DOI: 10.1016/j.humpath.2017.01.016

Cholangiolocellular carcinoma is a type of intrahepatic cholangiocarcinoma (ICC). According to the 2010 World Health Organization classification, this carcinoma is a combined hepatocellular-cholangiocarcinoma with stem cell features, cholangiolocellular type (CHC-SC-CLC). The aim of this study was to compare the clinicopathological characteristics of CHC-SC-CLC and conventional ICC. Based on the gross and histologic characteristics, we divided consecutive ICC tumors into CHC-SC-CLC (n = 23), mass-forming (MF; n = 57), and non-MF (n = 22) groups. Compared with MF and non-MF groups, the CHC-SC-CLC group featured history of hepatolithiasis or bile duct operation in significantly fewer patients (4.3% versus 14.8% and 86.4%, respectively; P < .001) and was more common in the right lobe (70% versus 47% and 27%; P = .033) but lower frequency of invasive growth or peritumoral Glisson sheath invasion (61% and 22% versus 77% and 33% and 100% and 86%, respectively; P = .002 and P < .001) and absence of mucous production (0 versus 77% and 96%; P < .001). In CHC-SC-CLCs, the mutation rate of isocitrate dehydrogenase 1 (IDH1) or IDH2 was significantly higher (35%) than in MF (4%) or non-MF (0) ICCs (P < .001). The 1-, 3-, and 5-year postresection survival rates were also significantly better with CHC-SC-CLCs (93%, 79%, and 52%, respectively) than with MF (72%, 46%, and 40%) or non-MF (61%, 18%, and 0) ICCs (P = .041). Thus, CHC-SC-CLC tumors demonstrated an indolent growth pattern, more frequent IDH1/2 gene mutations, and better prognosis than did MF or non-MF ICC tumors. •CHC-SC-CLC was commonly related to prior liver injury but not cholangiolithiasis or cholangitis.•CHC-SC-CLC demonstrated indolent growth characteristics, for example, fewer Glisson sheath invasions.•IDH1/2 mutations were detected in a third of CHC-SC-CLCs but few ICCs.•CHC-SC-CLC showed a better postresection prognosis than did ICC.