Cancer Surveillance in Patients with Primary Sclerosing Cholangitis

Overview

Journal Hepatology

Specialty Gastroenterology

Date 2011 Jul 28

PMID 21793028

Citations 99

Authors

Nataliya Razumilava

Gregory J Gores

Keith D Lindor

Affiliations

Soon will be listed here.

Abstract

Primary sclerosing cholangitis (PSC) is a chronic fibroinflammatory syndrome involving the biliary tract, often accompanied by inflammatory bowel disease (IBD). This syndrome is a prototype disease linking chronic inflammation to carcinogenesis. Indeed, PSC is associated with an increased risk of cholangiocarcinoma (CCA), gallbladder cancer, hepatocellular carcinoma (HCC), and colorectal cancer. Herein, we review the risk for these malignancies in PSC and discuss rational cancer surveillance strategies for these patients. Where evidence is limited, we suggest a pragmatic approach. In this regard, we recommend interval screening for CCA with noninvasive imaging modalities and serum carbohydrate antigen 19-9 determinations annually. These imaging studies also serve to screen for gallbladder cancer and HCC. Screening for colorectal cancer is more firmly established in PSC patients with IBD and includes colonoscopy at the time of PSC diagnosis and, thereafter, at 1-2-year intervals. We also highlight areas where more information is required, such as management of biliary tract dysplasia and cancer chemoprevention in PSC.

Citing Articles

Exhaled volatile organic compounds for cholangiocarcinoma diagnosis.

Siriwong N, Sukaram T, Tansawat R, Apiparakoon T, Tiyarattanachai T, Marukatat S Liver Res. 2025; 6(3):191-197.

PMID: 39958199 PMC: 11791817. DOI: 10.1016/j.livres.2022.09.001.

Treatment of cholangiocarcinoma in patients with primary sclerosing cholangitis: a comprehensive review.

Villard C, Jorns C, Bergquist A eGastroenterology. 2025; 2(1):e100045.

PMID: 39944751 PMC: 11770459. DOI: 10.1136/egastro-2023-100045.

Racial Disparities in Inpatient Hospital Outcomes of Primary Sclerosing Cholangitis in United States: Nationwide Analysis.

Vohra I, Gopakumar H, Dahiya D, Kahaleh M, Sharma N Diagnostics (Basel). 2024; 14(22).

PMID: 39594159 PMC: 11592423. DOI: 10.3390/diagnostics14222493.

The Constellation of Risk Factors and Paraneoplastic Syndromes in Cholangiocarcinoma: Integrating the Endocrine Panel Amid Tumour-Related Biology (A Narrative Review).

Ciobica M, Sandulescu B, Chicea L, Iordache M, Groseanu M, Carsote M Biology (Basel). 2024; 13(9).

PMID: 39336089 PMC: 11429066. DOI: 10.3390/biology13090662.

Still elusive: Developments in the accurate diagnosis of indeterminate biliary strictures.

Affarah L, Berry P, Kotha S World J Gastrointest Endosc. 2024; 16(6):297-304.

PMID: 38946851 PMC: 11212512. DOI: 10.4253/wjge.v16.i6.297.

References

Zielinski M, Atwell T, Davis P, Kendrick M, Que F . Comparison of surgically resected polypoid lesions of the gallbladder to their pre-operative ultrasound characteristics. J Gastrointest Surg. 2008; 13(1):19-25. DOI: 10.1007/s11605-008-0725-2. View

Malik I . Gallbladder cancer: current status. Expert Opin Pharmacother. 2004; 5(6):1271-7. DOI: 10.1517/14656566.5.6.1271. View

Kingham J, Kochar N, Gravenor M . Incidence, clinical patterns, and outcomes of primary sclerosing cholangitis in South Wales, United Kingdom. Gastroenterology. 2004; 126(7):1929-30. DOI: 10.1053/j.gastro.2004.04.052. View

Nakanuma Y, Harada K, Ishikawa A, Zen Y, Sasaki M . Anatomic and molecular pathology of intrahepatic cholangiocarcinoma. J Hepatobiliary Pancreat Surg. 2003; 10(4):265-81. DOI: 10.1007/s00534-002-0729-3. View

Faubion Jr W, Loftus E, Sandborn W, Freese D, Perrault J . Pediatric "PSC-IBD": a descriptive report of associated inflammatory bowel disease among pediatric patients with psc. J Pediatr Gastroenterol Nutr. 2001; 33(3):296-300. DOI: 10.1097/00005176-200109000-00013. View

Lazcano-Ponce E, Miquel J, Munoz N, Herrero R, Ferrecio C, Wistuba I . Epidemiology and molecular pathology of gallbladder cancer. CA Cancer J Clin. 2002; 51(6):349-64. DOI: 10.3322/canjclin.51.6.349. View

Kopans D . The 2009 US Preventive Services Task Force (USPSTF) guidelines are not supported by science: the scientific support for mammography screening. Radiol Clin North Am. 2010; 48(5):843-57. DOI: 10.1016/j.rcl.2010.06.005. View

Lindkvist B, Benito de Valle M, Gullberg B, Bjornsson E . Incidence and prevalence of primary sclerosing cholangitis in a defined adult population in Sweden. Hepatology. 2010; 52(2):571-7. DOI: 10.1002/hep.23678. View

Helzberg J, Petersen J, Boyer J . Improved survival with primary sclerosing cholangitis. A review of clinicopathologic features and comparison of symptomatic and asymptomatic patients. Gastroenterology. 1987; 92(6):1869-75. DOI: 10.1016/0016-5085(87)90618-4. View

10.

Yamamoto T, Uki K, Takeuchi K, Nagashima N, Honjo H, Sakurai N . Early gallbladder carcinoma associated with primary sclerosing cholangitis and ulcerative colitis. J Gastroenterol. 2003; 38(7):704-6. DOI: 10.1007/s00535-002-1126-z. View

11.

Chapman R, Fevery J, Kalloo A, Nagorney D, Boberg K, Shneider B . Diagnosis and management of primary sclerosing cholangitis. Hepatology. 2010; 51(2):660-78. DOI: 10.1002/hep.23294. View

12.

Sinakos E, Saenger A, Keach J, Kim W, Lindor K . Many patients with primary sclerosing cholangitis and increased serum levels of carbohydrate antigen 19-9 do not have cholangiocarcinoma. Clin Gastroenterol Hepatol. 2011; 9(5):434-9.e1. DOI: 10.1016/j.cgh.2011.02.007. View

13.

Rudolph G, Gotthardt D, Kloeters-Plachky P, Rost D, Kulaksiz H, Stiehl A . In PSC with dominant bile duct stenosis, IBD is associated with an increase of carcinomas and reduced survival. J Hepatol. 2010; 53(2):313-7. DOI: 10.1016/j.jhep.2010.02.030. View

14.

Silveira M, Lindor K . Primary sclerosing cholangitis. Can J Gastroenterol. 2008; 22(8):689-98. PMC: 2661291. DOI: 10.1155/2008/824168. View

15.

Leidenius M, Farkkila M, Karkkainen P, Taskinen E, Kellokumpu I, Hockerstedt K . Colorectal dysplasia and carcinoma in patients with ulcerative colitis and primary sclerosing cholangitis. Scand J Gastroenterol. 1997; 32(7):706-11. DOI: 10.3109/00365529708996522. View

16.

Tung B, Emond M, Haggitt R, Bronner M, Kimmey M, Kowdley K . Ursodiol use is associated with lower prevalence of colonic neoplasia in patients with ulcerative colitis and primary sclerosing cholangitis. Ann Intern Med. 2001; 134(2):89-95. DOI: 10.7326/0003-4819-134-2-200101160-00008. View

17.

Fong Y, Wagman L, Gonen M, Crawford J, Reed W, Swanson R . Evidence-based gallbladder cancer staging: changing cancer staging by analysis of data from the National Cancer Database. Ann Surg. 2006; 243(6):767-71. PMC: 1570569. DOI: 10.1097/01.sla.0000219737.81943.4e. View

18.

Ang T, Fock K, Ng T, Teo E, Chua T, Tan J . Clinical profile of primary sclerosing cholangitis in Singapore. J Gastroenterol Hepatol. 2002; 17(8):908-13. DOI: 10.1046/j.1440-1746.2002.02835.x. View

19.

Sharma R, London M, Magenta L, Posner M, Roggin K . Preemptive surgery for premalignant foregut lesions. J Gastrointest Surg. 2009; 13(10):1874-87. DOI: 10.1007/s11605-009-0935-2. View

20.

Boberg K, Aadland E, Jahnsen J, Raknerud N, Stiris M, Bell H . Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population. Scand J Gastroenterol. 1998; 33(1):99-103. DOI: 10.1080/00365529850166284. View