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PSC-Associated Cholangiocarcinoma: Diagnostic and Therapeutic Considerations

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Diagnosis and Management of Cholangiocarcinoma

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Abstract

Primary sclerosing cholangitis (PSC) is a chronic inflammatory liver disease, of unknown etiology, targeting cholangiocytes in any portion of the biliary system. Cholangiocarcinoma (CCA) is a feared complication of PSC, with a lifetime risk of 7–14%, no matter the duration of underlying PSC. Since a prominent fibrotic reaction closely abutting the bile ducts is a common trait of both PSC and CCA, PSC may be regarded as paradigmatic of the pathogenetic sequence leading from chronic inflammatory epithelial damage to malignant transformation. The risk of CCA is a major challenge in the management of PSC, raising several questions about disease surveillance, early tumor detection, prevention, and treatment. Herein, we discuss the critical issues of PSC-associated CCA, highlighting the need for a multimodal approach where recent advances in imaging studies, endoscopic procedures, and novel tumoral biomarkers are emphasized.

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Abbreviations

AIH:

Autoimmune hepatitis

ALP:

Alkaline phosphatase

CA:

Carbohydrate antigen

CCA:

Cholangiocarcinoma

CD:

Crohn’s disease

COX:

Cyclooxygenase

CT:

Computer tomography

DWI:

Diffusion-weighted imaging

ECM:

Extracellular matrix

ERCP:

Endoscopic retrograde cholangiopancreatography

EUS:

Endoscopic ultrasound

EV:

Extracellular vesicle

F-FDG:

F-Fluoro-deoxyglucose

FISH:

Fluorescence in situ hybridization

FNA:

Fine needle aspiration

FUT:

Fucosyltransferase

GP2:

Glycoprotein 2

HCC:

Hepatocellular carcinoma

IBD:

Inflammatory bowel disease

lncRNA:

Long noncoding RNA

LT:

Liver transplantation

miRNA:

Micro-ribonucleic acid

MRCP:

Magnetic resonance cholangiopancreatography

MRI:

Magnetic resonance imaging

NO:

Nitric oxide

pCCA:

Perihilar cholangiocarcinoma

PET:

Positron emission tomography

PSC:

Primary sclerosing cholangitis

TRAIL:

Tumor necrosis factor-related apoptosis-inducing ligand

UC:

Ulcerative colitis

US:

Ultrasonography

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Authors and Affiliations

  1. Department of Molecular Medicine, University of Padua School of Medicine, Padua, Italy

    Silvia Cagnin & Luca Fabris

  2. Division of Gastroenterology, Department of Medicine, Olive View-UCLA Medical Center, Sylmar, CA, USA

    James H. Tabibian

  3. David Geffen School of Medicine at UCLA, Los Angeles, CA, USA

    James H. Tabibian

  4. Digestive Disease Section, Liver Center, Yale University, New Haven, CT, USA

    Luca Fabris

Authors
  1. Silvia Cagnin
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  2. James H. Tabibian
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  3. Luca Fabris
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Correspondence to Luca Fabris .

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Editors and Affiliations

  1. Division of Gastroenterology, Department of Medicine, Olive View-UCLA Medical Center, Sylmar, CA, USA

    James H. Tabibian

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Cagnin, S., Tabibian, J.H., Fabris, L. (2021). PSC-Associated Cholangiocarcinoma: Diagnostic and Therapeutic Considerations. In: Tabibian, J.H. (eds) Diagnosis and Management of Cholangiocarcinoma. Springer, Cham. https://doi.org/10.1007/978-3-030-70936-5_10

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  • DOI: https://doi.org/10.1007/978-3-030-70936-5_10

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