Malignancy and Primary Sclerosing Cholangitis: Cholangiocarcinoma, Hepatocellular Carcinoma, and Gallbladder Carcinoma
Primary sclerosing cholangitis is associated with an increased risk of malignancy. The chronic inflammation of the intra- and extrahepatic biliary tract predisposes to the development of cholangiocarcinoma and gallbladder carcinoma. In those who progress
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Malignancy and Primary Sclerosing Cholangitis: Cholangiocarcinoma, Hepatocellular Carcinoma, and Gallbladder Carcinoma Larissa Muething and James R. Burton Jr.
Technical Terms and Abbreviations AASLD AFP AJCC BCLC CCA CLIP CT CTP DDLT ERCP EUS FISH FNA GBC HCC LDLT MELD MRCP MRI
American Association for the Study of Liver Diseases Alpha-fetoprotein American Joint Committee on Cancer Barcelona Clinic Liver Cancer Cholangiocarcinoma Cancer of the Liver Italian Program Computerized tomography Child-Turcotte-Pugh Deceased donor liver transplantation Endoscopic retrograde cholangiopancreatography Endoscopic ultrasound Fluorescence in situ hybridization Fine needle aspiration Gallbladder carcinoma Hepatocellular carcinoma Living donor liver transplantation Model for end-stage liver disease Magnetic resonance cholangiopancreatography Magnetic resonance imaging
L. Muething (*) • J.R. Burton Jr. University of Colorado Hospital, Anschutz Outpatient Pavilion, 7th Floor, Transplant Center, 1635 Aurora Court, B154, Aurora, CO 80045, USA e-mail: [email protected]
NCCN
National Comprehensive Cancer Network OLT Orthotopic liver transplantation PDT Photodynamic therapy PIVKA II Prothrombin induced by vitamin K absence II PSC Primary sclerosing cholangitis RFA Radiofrequency ablation TACE Transarterial chemoembolization TNM Tumor, node, metastasis UCSF University of California, San Francisco UNOS United Network for Organ Sharing US Ultrasound Y-90 Yttrium-90
Cholangiocarcinoma Introduction Cholangiocarcinoma (CCA) is a common and devastating malignancy associated with primary sclerosing cholangitis (PSC). Cholangiocarcinoma is classified into intrahepatic CCA and extrahepatic CCA. Intrahepatic cholangiocarcinomas are located within the hepatic parenchyma. The anatomic boundary between intrahepatic CCAs and extrahepatic CCAs are the second-order bile ducts. Extrahepatic CCA is further differentiated into perihilar tumors, also known as Klatskin tumors, and distal tumors. The cystic ducts serve
© Springer International Publishing Switzerland 2017 L.M. Forman (ed.), Primary Sclerosing Cholangitis, DOI 10.1007/978-3-319-40908-5_2
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L. Muething and J.R. Burton Jr.
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as the anatomic boundary between perihilar and distal tumors. The location of CCA affects both the management and prognosis. The majority of CCAs associated with PSC are perihilar. Overall CCA has a poor prognosis in PSC.
Epidemiology Individuals with PSC are at significantly higher risk for developing CCA. Bergquist et al. found that in a Swedish cohort, the incidence of hepatobiliary malignancy was 161 times higher in individuals with PSC compared to the general population [5]. The incidence of CCA in PSC reported in the literature varies widely but is most frequently reported to be in the range 7–14 % in population-based studies [5, 12, 38]. A higher incidence is reported in transplant studies with 10–36 % of incidental diagnoses of CCA at the time of transplant for PSC [1, 27, 34, 49, 52]. Up to 50 % of cases of cholangiocarcinoma are diagnosed with
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