Hyperammonemic Encephalopathy: A Rare Presentation of Fibrolamellar Hepatocellular Carcinoma

• DOI: 10.1097/maj.0b013e3181bccfb4
• Publication Date: 2009-12-09T08:30:17Z
• Authors: Saurabh Sethi; Nishant Tageja; Maneesh Dave; Apurva Badheka; Sanjay Revankar; Haitham Arabi; Jatinder Singh
• Keywords: Male; 0301 basic medicine; 03 medical and health sciences; Carcinoma, Hepatocellular; Adolescent; Liver Neoplasms; Humans; Hyperammonemia; Neurotoxicity Syndromes; 3. Good health

Fibrolamellar carcinoma (FLC) is a rare malignant hepatocellular tumor of unknown etiology, arising almost exclusively from noninfected, noncirrhotic liver of young adults. FLC has traditionally been considered to have better survival than hepatocellular carcinoma; however, this notion might be highly erroneous. Patients with metastatic disease at presentation have a dismal prognosis with 5-year survival of only 15%. We describe a case of highly aggressive metastatic FLC that presented as hyperammonemic encephalopathy, which has never been previously reported in the literature.