Primary sclerosing cholangitis (PSC) is a rare progressive liver disease that damages bile ducts and significantly increases the risk of bile duct cancer, particularly a type called cholangiocarcinoma (CCA). This cancer is aggressive, and curative surgery is uncommon. Liver transplantation is a potential treatment option for some PSC-CCA patients, especially if the cancer is caught early. Early diagnosis is essential for successful treatment.

PSC can affect people of all ages but primarily strikes men in their 30s and 40s. It is often accompanied by inflammatory bowel disease (IBD). The disease can progress to liver failure and increase the risk of colorectal cancer as well. There is no cure for PSC, but medications can help manage symptoms.

Diagnosing CCA in PSC patients is difficult due to limitations in diagnostic tools and the non-specific symptoms, which often appear late and mimic those of progressive PSC itself. Magnetic resonance imaging with magnetic resonance cholangiopancreatography (MRI/MRCP) is the best imaging modality for suspected PSC-CCA.

For a select group of patients with PSC-CCA, liver transplantation has emerged as a potentially curative treatment, particularly for a specific type of bile duct cancer called perihilar CCA (pCCA). However, most patients are diagnosed at an advanced stage when curative options are no longer available.

Due to the challenges in diagnosing CCA early, a low threshold for suspicion is recommended in high-risk PSC patients. Regular high-quality MRI/MRCP scans are crucial for early detection.

Researchers are actively seeking better strategies for early detection of CCA in PSC patients. Developing more advanced surveillance techniques and diagnostic tests is a high priority.

PSC is a severe liver disease that significantly increases the risk of bile duct cancer. Early detection of CCA is essential for successful treatment with liver transplantation. More research is needed to improve early detection methods and expand treatment options for PSC patients.

See the article:

Villard C, Jorns C, Bergquist A. Treatment of cholangiocarcinoma in patients with primary sclerosing cholangitis: a comprehensive review. eGastroenterology 2024;2:e100045. doi:10.1136/egastro-2023-100045

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