Walter Reed National Military Medical Center Hanover, PA, United States
Azfar S. Syed, DO, MBA, Jared S. Magee, DO, MPH, Dawn Torres, MD Walter Reed National Military Medical Center, Bethesda, MD
Introduction: Cholangiocarcinoma (CCA) is a rare cancer arising from the epithelial cells of the biliary tree that is associated with a high mortality. CCA has traditionally been categorized as intrahepatic (iCCA) or extrahepatic (eCCA) based on site of involvement. Primary sclerosing cholangitis, choledochal cysts and cirrhosis have been established as risk factors for CCA. Non-alcoholic steatohepatitis (NASH), has been suggested as a risk factor for CCA but data is limited. Two presentations of CCA in non-cirrhotic NASH are discussed here.
Case Description/Methods: Case 1: 60 year old obese male with history of biopsy confirmed NASH underwent repeat liver biopsy for clinical trial enrollment, which confirmed steatohepatitis without cirrhosis. Post-procedure the patient experienced persistent right upper quadrant pain that prompted an emergency room (ER) visit, where a CT abdomen with contrast showed a non-specific hypodensity with possible retroperitoneal lymphadenopathy. Subsequent MRI and EUS confirmed a 4.3cm, stage IV Klatskin tumor and a serum cancer antigen (CA) 19-9 level of 1823 U/mL. The patient was referred for neoadjuvant chemotherapy with the goal of eventual surgical resection.
Case 2: 72 year old obese female with known NASH was evaluated in the ER for atypical chest pain in the setting of 50 pounds of intentional weight loss in the preceding year. CT Angiography obtained at the time to rule out an aortic dissection revealed 8.8cm right hepatic lobe lesion. Subsequent MRI demonstrated steatosis without cirrhosis and a large heterogeneously enhancing lesion with peripheral rim enhancement and central necrosis that was highly suggestive of iCCA. CA-19-9 was noted to be 100 U/mL. She underwent a right hepatectomy with final pathology revealing iCCA and NASH.
Discussion: These cases demonstrate the occurrence of CCA in non-cirrhotic NASH. Metabolic syndrome is a risk factor for intra-hepatic CCA so this may explain the association of CCA and non-cirrhotic NASH. It is thought that this process may be driven from the NASH inflammatory state or from disruption in the gut microbiome. iCCA is more common than eCCA which supports its likely shared origin with HCC. Given the increasing prevalence of NASH, it is important for clinicians to be aware of this rare but morbid association.
The views expressed are those of the author(s) and do not reflect the official policy of the Department of the Army, the Department of Defense or the U.S. Government.
Azfar Syed indicated no relevant financial relationships.
Jared Magee indicated no relevant financial relationships.
Dawn Torres indicated no relevant financial relationships.
Azfar S. Syed, DO, MBA, Jared S. Magee, DO, MPH, Dawn Torres, MD. P1896 - Perils of Fatty Liver Disease: Two Cases of Cholangiocarcinoma in Non-Cirrhotic NASH, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.