Cholangiocarcinoma: Classification, Histopathology and Molecular Carcinogenesis

Gábor Lendvai, Tímea Szekerczés, Idikó Illyés, Réka Dóra, Endre Kontsek, Alíz Gógl, András Kiss, Klára Werling, Ilona Kovalszky, Zsuzsa Schaff, Katalin Borka

Research output: Contribution to journalReview article

7 Citations (Scopus)


Cholangiocarcinoma (CC) is the second most common tumor of the liver, originating from the biliary system with increasing incidence and mortality worldwide. Several new classifications review the significance of tumor localization, site of origin, proliferation and biomarkers in the intrahepatic, perihilar and distal forms of the lesion. Based on growth pattern mass-forming, periductal-infiltrating, intraductal, undefined and mixed types are differentiated. There are further subclassifications which are applied for the histological features, in particular for intrahepatic CC. Recognition of the precursors and early lesions of CC including biliary intraepithelial neoplasia (BilIN), intraductal papillary neoplasm of the bile ducts (IPNB), biliary mucinous cystic neoplasm (MCNB) and the candidate precursors, such as bile duct adenoma and von Meyenburg complex is of increasing significance. In addition to the previously used biliary markers detected by immunohistochemistry, several new markers have been added to the differentiation of both the benign and malignant lesions, which can be used to aid in the subclassification in association with the outcome of CC. Major aspects of biliary carcinogenesis have been revealed, yet, the exact way of this diverse process is still unclear. The factors contributing to molecular cholangiocarcinogenesis include various risk factors, different anatomical localizations, multiple cellular origins, genetic and epigenetic alterations, tumor microenvironment, heterogeneity and clonal evolution. Driver mutations have been identified, implying that they are optimal candidates for targeted therapy. The most promising therapeutic candidates have entered clinical trials.

Original languageEnglish
Pages (from-to)3-15
Number of pages13
JournalPathology and Oncology Research
Issue number1
Publication statusPublished - Jan 1 2020


  • Biliary markers
  • Cholangiocarcinoma
  • Liver cancer
  • MicroRNA
  • Stem cells

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Oncology
  • Cancer Research

Fingerprint Dive into the research topics of 'Cholangiocarcinoma: Classification, Histopathology and Molecular Carcinogenesis'. Together they form a unique fingerprint.

  • Cite this

    Lendvai, G., Szekerczés, T., Illyés, I., Dóra, R., Kontsek, E., Gógl, A., Kiss, A., Werling, K., Kovalszky, I., Schaff, Z., & Borka, K. (2020). Cholangiocarcinoma: Classification, Histopathology and Molecular Carcinogenesis. Pathology and Oncology Research, 26(1), 3-15.