Walter Reed National Military Medical Center Bethesda, MD, United States
Benjamin T. Wilson, MD, John W. Lee, MD, Dawn Torres, MD Walter Reed National Military Medical Center, Bethesda, MD
Introduction: Cholangiocarcinoma (CCA) is an uncommon cancer of the biliary tree that occurs at increased incidence in certain chronic liver diseases including Primary Sclerosing Cholangitis (PSC). Patients with PSC have markedly increased rates of Inflammatory Bowel Disease (IBD), but having IBD alone is also considered a risk factor for CCA. This is a unique presentation of CCA in a patient with Crohn’s Disease (CD) in deep remission. At the time of presentation, he was also noted to have decompensated cirrhosis attributed to non-alcoholic fatty liver disease (NAFLD).
Case Description/Methods: A 57-year-old male with CD in remission on infliximab presented with new stigmata of cirrhosis. On MRI imaging, he was found to have a liver mass concerning for a primary hepatobiliary malignancy within a cirrhotic appearing liver without findings suggestive of PSC. Evaluation from years prior was notable for a negative serologic work-up for a chronic intermittent mild elevation of his transaminases with clinical notes suggesting the elevation was due to NAFLD. Tumor markers were notable for carbohydrate antigen 19-9 (CA19-9) 32 U/mL, a carcinoembryonic antigen (CEA) 5.5 ng/mL, and an alpha-1-fetoprotein (AFP) of 174 ng/mL. Biopsy of the liver mass demonstrated poorly differentiated adenocarcinoma with specialty stains suggesting CCA. He was started on palliative chemotherapy and targeted therapy with olaparib.
Discussion: CCA is a highly fatal tumor and is the second most common primary hepatic malignancy. Risk factors include IBD, hepatitis B and C viruses, cirrhosis, diabetes mellitus, obesity, alcohol consumption, tobacco smoking, and parasitic infection. PSC is the main risk factor in the United States. Postulated mechanisms of carcinogenesis include chronic inflammation, proliferation of biliary epithelium, production of endogenous bile mutagens, and bile stasis. Interestingly this patient did not have PSC although he did have cirrhosis and IBD. He also had an elevated AFP which suggested a diagnosis of Hepatocellular Carcinoma (HCC). Liver biopsy was performed to clarify the diagnosis as imaging was atypical for HCC. This case demonstrates a unique presentation of CCA in the background of quiescent CD and undiagnosed cirrhosis and highlights the limitations of imaging and the currently available tumor markers for hepatobiliary malignancies.
Benjamin Wilson indicated no relevant financial relationships.
John Lee indicated no relevant financial relationships.
Dawn Torres indicated no relevant financial relationships.
Benjamin T. Wilson, MD, John W. Lee, MD, Dawn Torres, MD. P0105 - Cholangiocarcinoma in IBD Without PSC, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.