Updates in the diagnosis of combined hepatocellular-cholangiocarcinoma

• Published in: Human pathology Vol. 96; pp. 48 - 55
• Main Authors: Sciarra, Amedeo, Park, Young Nyun, Sempoux, Christine
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-02-2020; Elsevier Limited
• Subjects: Biomarkers; Cholangiocarcinoma; Cholangiolocarcinoma; Classification; Combined hepatocellular-cholangiocarcinoma; Hepatocellular carcinoma; Hypotheses; Jargon; Liver cancer; Morphology; Primary liver carcinoma; Stem cells; Tumors; IHC; Combined hepatocellular-cholangiocarcinoma; cHCC-CCA; PLC; Primary liver carcinoma; Cholangiolocarcinoma; iCCA; HCC; Hepatocellular carcinoma; CLC; Cholangiocarcinoma
• ISSN: 0046-8177; 1532-8392
• DOI: 10.1016/j.humpath.2019.11.001

Combined hepatocellular-cholangiocarcinoma (cHCC-CCA) is a rare primary liver carcinoma showing variable degrees of differentiation toward hepatocellular and cholangiocellular carcinoma. Its great heterogeneity in term of morphology, immunophenotype, molecular, radiological and clinical features represents a challenge still to overcome. The multidisciplinary 2018 International Consensus on the nomenclature of cHCC-CCA allowed to define key issues of this entity. Here we review the historical controversies of cHCC-CCA, resume the key elements of the 2018 consensus, now incorporated in the 2019 WHO classification, and propose a short survival guide to help surgical pathologists facing cHCC-CCA in their routine workup.