Diffuse Liver Cholangiocarcinoma Presenting as Fulminant Liver Failure

Abstract

Introduction: Cryptogenic acute liver failure (ALF) can occur in up to 40 % of cases. There are few reports of diffuse liver tumors (primary or metastatic) leading to ALF. We herein report an unusual case of ALF secondary to diffuse cholangiocarcinoma. Case report: A 55-year-old woman was admitted to hospital with jaundice over the past 15 days, associated with general fatigue, nausea, vomiting and fever. During the admission the patient developed confusion, lactic acidosis and hypotension. Her past medical history revealed a cholecystectomy and myomectomy 10 months ago, without any history of hepatotoxic medication use. There were no others comorbidities. Laboratory evaluation included: TB 11.3 mg/dL; AST 4248 IU/L; GGTP 289 IU/L, and INR of 2.66. Hepatitis serologies were negative. Ultrasound and CT scan revealed: heterogeneous enlarged liver without any focal lesions and no signs of portal hypertension. Main portal and hepatic veins were patent. Based on clinical and laboratory evaluation, patient underwent to liver transplantation 48 hours after ICU admission. Intraoperative liver macroscopy was compatible with ALF. Explanted liver revealed a diffuse poorly differentiated liver cholangiocarcinoma with multiple microvascular tumoral emboli. There were no signs of underlying disease (i.e. cirrhosis). Immunostaining for both cytokeratins 7 and 19 were positive. Conclusion: To the best of our knowledge this is the first report of ALF associated with cholangiocarcinoma. Neoplastic involvement of liver should be considered in the differential diagnosis of ALF of unknown etiology. Pre-transplant diagnosis should be always pursued due to the dismal prognosis of these conditions. Given the limitation of imaging studies in this setting, liver biopsy can be an option to obtain an accurate diagnosis.