Risk of Hepatobiliary-Gastrointestinal Malignancies and Appropriate Cancer Surveillance in Patients With Primary Sclerosing Cholangitis.

Sujani Yadlapati,Thomas A Judge

doi:10.7759/cureus.19922

Abstract

Patients with primary sclerosing cholangitis (PSC) are at risk of hepatobiliary and gastrointestinal cancers. Increased risk of cancer is a result of the chronic, progressive fibro-inflammatory state which ultimately results in the destruction of biliary ducts. PSC is often associated with inflammatory bowel disease (IBD). Patients with PSC are at significant risk of cholangiocarcinoma (CCA), gall bladder malignancy and those with IBD are at increased risk of colorectal cancer. It is important to implement cancer surveillance protocols in these patients. The aim of these protocols is the prevention or early detection of cancerous or pre-cancerous lesions. Given that PSC is rare, large prospective studies evaluating the risk of malignancy in these patients are not available. A great deal of uncertainty exists regarding how to best implement cancer surveillance in these patients. About 50% of deaths in PSC patients are due to malignancy and many patients eventually progress to end-stage liver disease and succumb to hepatic failure. In this review, we cover cancer surveillance strategies in PSC patients based on existing literature and expert opinions.

Highlights

BackgroundPrimary sclerosing cholangitis (PSC) is characterized by chronic hepatobiliary inflammation that causes stricturing disease in the biliary tree
Patients with primary sclerosing cholangitis (PSC) are at significant risk of cholangiocarcinoma (CCA), gall bladder malignancy and those with inflammatory bowel disease (IBD) are at increased risk of colorectal cancer
Patients with PSC are at high risk of hepatobiliary cancers and patients with both PSC and IBD (PSC-IBD) have an elevated risk of colon cancer compared to patients with IBD alone [5]

Summary

Introduction

Primary sclerosing cholangitis (PSC) is characterized by chronic hepatobiliary inflammation that causes stricturing disease in the biliary tree. Primary sclerosing cholangitis patients are at an increased risk of gall bladder malignancy. For patients with PSC alone, in the absence of IBD, some experts recommend colonoscopy at 3-5-year intervals [14,16] It is unclear if use of chromoendoscopy with targeted biopsies is superior to high-definition colonoscopy in these cases. One study reported by Bergquist, and colleagues noted a risk of pancreatic cancer up to 14 times higher in PSC patients than in the general population [7]. CCA: cholangiocarcinoma; HCC: hepatocellular carcinoma; PSC: primary sclerosing cholangitis; MRI: magnetic resonance imaging; MRCP: magnetic resonance cholangio-pancreatography; US: ultrasound. PSC: primary sclerosing cholangitis; CCA: cholangiocarcinoma; CRC: colorectal cancer; MRI: magnetic resonance imaging; MRCP: magnetic resonance cholangio-pancreatography; US: ultrasound; LGD: low-grade dysplasia; HGD: high-grade dysplasia

Conclusions

Findings

Disclosures