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Current Hepatology Reports

, Volume 18, Issue 4, pp 408–416 | Cite as

Screening Indications and Treatments for Cholangiocarcinoma

  • Joseph C. Ahn
  • Ju Dong YangEmail author
Hepatic Cancer (N Parikh, Section Editor)
  • 22 Downloads
Part of the following topical collections:
  1. Topical Collection on Hepatic Cancer

Abstract

Purpose of Review

The goal of this review paper is to provide a comprehensive overview of cholangiocarcinoma (CCA) including its classification, epidemiology, risk factors, surveillance, diagnosis, and treatment.

Recent Findings

Guidelines recommend CCA surveillance in PSC patients with MRI/MRCP or ultrasound and CA 19-9 every 6–12 months. Fluorescience in situ hybridization and next-generation sequencing improve the poor sensitivity of biliary brushings. Surgical resection with negative margins gives the best chance of survival, and liver transplantation is an option for patients with very early intrahepatic CCA and perihilar CCA.

Summary

CCA is a deadly epithelial malignancy of the biliary-ductal system and is the second most common primary liver cancer. Surveillance should be offered to all patients with PSC. CCA carries poor prognosis, especially if resection or liver transplantion is not feasible. Novel biomarkers and therapeutic options such as molecularly targeted therapy and immunotherapy hold promise to improve the detection and outcome of CCA patients.

Keywords

Cholangiocarcinoma Primary sclerosing cholangitis Surveillance MRI/MRCP CA 19-9 ERCP Biliary brushing R0 resection Chemotherapy Locoregional therapy Liver transplantation 

Abbreviations

ACG

American College of Gastroenterology

AGA

American Gastroenterological Association

ALPPS

Associating liver partition and portal vein ligation for staged hepatectomy

CA 19-9

Carbohydrate antigen 19-9

CASCAP

Cholangiocarcinoma Screening and Care Program

CCA

Cholangiocarcinoma

cHCC-CCA

Combined hepatocellular-cholangiocarcinoma

dCCA

Distal cholangiocarcinoma

ERCP

Endoscopic retrograde cholangiopancreatography

EUS

Endoscopic ultrasound

FISH

Fluorescence in situ hybridization

FLR

Future liver remnant

FNA

Fine-needle aspiration

HAIP

Hepatic artery infusion pump

iCCA

Intrahepatic cholangiocarcinoma

MRCP

Magnetic resonance cholangiopancreatography

MRI

Magnetic resonance imaging

NGS

Next-generation sequencing

pCCA

Perihilar cholangiocarcinoma

PSC

Primary sclerosing cholangitis

PVE

Portal vein embolization

SEER

Surveillance, Epidemiology, and End Results

TACE

Transarterial chemoembolization

TARE

Transarterial radioembolization

Notes

Compliance with Ethical Standards

Conflict of Interest

Joseph C. Ahn and Ju Dong Yang each declare no potential conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

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Authors and Affiliations

  1. 1.Division of Gastroenterology and HepatologyMayo ClinicRochesterUSA
  2. 2.Division of Digestive and Liver Diseases, Department of MedicineCedars Sinai Medical CenterLos AngelesUSA
  3. 3.Comprehensive Transplant CenterCedars Sinai Medical CenterLos AngelesUSA
  4. 4.Samuel Oschin Comprehensive Cancer InstituteCedars Sinai Medical CenterLos AngelesUSA

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