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A Contemporary Approach to Diagnosis and Treatment of Combined Hepatocellular-Cholangiocarcinoma

  • Hepatic Cancer (N Parikh, Section Editor)
  • Published:
Current Hepatology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

To provide updates on terminology, epidemiology, diagnosis, and treatment of combined hepatocellular-cholangiocarcinoma (cHCC-CCA).

Recent Findings

cHCC-CCAs are tumors that in the same nodule contain a variable degree of HCC and CCA components with a transition zone. cHCC-CCAs develop in cirrhotic and non-cirrhotic livers like and is associated with poor outcomes. Mutations in TP53, TERT promoter, and ARID1A are the most common genetic aberrations in cHCC-CCA. Fusion gene PTMS-AP1G1 is unique for cHCC-CCA. A biopsy is required for diagnosis. Surgical resection remains treatment of choice, while liver transplantation for early cHCC-CCA is associated with favorable outcomes. Gemcitabine-based therapy shows benefits for advanced cHCC-CCA.

Summary

cHCC-CCAs are a heterogeneous group of primary liver cancers with unique biological behavior. Multicenter studies are required for a molecular analysis to inform novel therapeutic approaches, and understand epidemiology and benefits of liver transplantation, liver-directed, and targeted therapies for this rare aggressive cancer.

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Abbreviations

cHCC-CCA:

Combined hepatocellular-cholangiocarcinoma

iCCA:

Intrahepatic cholangiocarcinoma

WHO:

World Health Organization

MRI:

Magnetic resonance imaging

PECAR:

Prognostic estimation of cHCC-CCA after resection

LN:

Lymph node

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Funding

This work was supported by NIDDK K08 DK122114-01 (NR); Gilead Research Scholar Award (NR).

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Authors and Affiliations

  1. Department of Internal Medicine, Division of Gastroenterology and Hepatology, University of Michigan, 109 Zina Pitcher Pl., BSRB, 2062, Ann Arbor, MI, 48109, USA

    Olga Raevskaya & Nataliya Razumilava

  2. Department of Pathology, University of Michigan, Ann Arbor, MI, USA

    Henry Appelman

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  1. Olga Raevskaya
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  2. Henry Appelman
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Correspondence to Nataliya Razumilava.

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Olga Raevskaya, Henry Appelman, and Natalita Razumilava declare that they have no conflict of interest.

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Raevskaya, O., Appelman, H. & Razumilava, N. A Contemporary Approach to Diagnosis and Treatment of Combined Hepatocellular-Cholangiocarcinoma. Curr Hepatology Rep 19, 478–485 (2020). https://doi.org/10.1007/s11901-020-00556-4

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