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Purpose of Review <p>This review aims to summarize the available literature on malignancy screening in patients with primary sclerosing cholangitis (PSC).</p> Recent findings <p>Primary sclerosing cholangitis (PSC) is a chronic, slowly progressive inflammatory disease of both the large and small bile ducts that ultimately leads to biliary fibrosis, cholestatic liver injury, and cirrhosis.</p> <p>Given its chronic inflammatory nature, PSC is associated with malignancy. PSC is associated with an 160-400 fold risk of cholangiocarcinoma and 9-78 fold risk of gallbladder cancer. If associated with inflammatory bowel disease, as is true in ~80% of people with PSC, then the risk of colorectal cancer is 10-fold increased- even in comparison to IBD. The risk of hepatocellular carcinoma is also high in the presence of PSC associated cirrhosis.</p> <p>Unfortunately, disease-modifying therapies for PSC do not exist, making symptomatic treatment and surveillance for malignancy the mainstay of clinical management. </p> Summary <p>In this review, we summarize the current clinical guidance on malignancy screening in PSC, highlight the gaps in current understanding, and emphasize the need for PSC-specific research moving forward to help more effectively manage this challenging disease and its myriad downstream sequelae.</p>

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Malignancy Screening in Primary Sclerosing Cholangitis

  • 期刊论文

  • 发表日期：

    2026年5月20日

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  • Bejan J. Saeedi,
  • Lisa M. Forman

摘要

Purpose of Review

This review aims to summarize the available literature on malignancy screening in patients with primary sclerosing cholangitis (PSC).

Recent findings

Primary sclerosing cholangitis (PSC) is a chronic, slowly progressive inflammatory disease of both the large and small bile ducts that ultimately leads to biliary fibrosis, cholestatic liver injury, and cirrhosis.

Given its chronic inflammatory nature, PSC is associated with malignancy. PSC is associated with an 160-400 fold risk of cholangiocarcinoma and 9-78 fold risk of gallbladder cancer. If associated with inflammatory bowel disease, as is true in ~80% of people with PSC, then the risk of colorectal cancer is 10-fold increased- even in comparison to IBD. The risk of hepatocellular carcinoma is also high in the presence of PSC associated cirrhosis.

Unfortunately, disease-modifying therapies for PSC do not exist, making symptomatic treatment and surveillance for malignancy the mainstay of clinical management.

Summary

In this review, we summarize the current clinical guidance on malignancy screening in PSC, highlight the gaps in current understanding, and emphasize the need for PSC-specific research moving forward to help more effectively manage this challenging disease and its myriad downstream sequelae.