Successful Liver Transplantation for Hyperammonemic Fibrolamellar Hepatocellular Carcinoma
نویسندگان
چکیده
Fibrolamellar hepatocellular carcinoma is a rare hepatocellular tumor usually arising in noninfected and noncirrhotic livers. Only 2 cases accompanied by hyperammonemia due to intrahepatic shunting have been reported. A 23-year-old white woman presented with a 2-week history of nausea, vomiting, generalized weakness, and intermittent right upper quadrant pain. Abdominal computerized tomography revealed a 13 x 9-cm hepatic mass. Core-needle biopsy revealed fibrolamellar hepatocellular carcinoma. She presented with coma due to hyperammonemia levels (peak at 437 mcg/dL) but without metastatic disease. She was urgently transplanted, started on daily sorafenib 8 weeks after transplantation, and was free of disease at 1 year after transplantation.
منابع مشابه
A Proposed Physiopathological Pathway to Hyperammonemic Encephalopathy in a Non-Cirrhotic Patient with Fibrolamellar Hepatocellular Carcinoma without Ornithine Transcarbamylase (OTC) Mutation
BACKGROUND Hyperammonemic encephalopathy is a potentially fatal condition that may progress to irreversible neuronal damage and is usually associated with liver failure or portosystemic shunting. However, other less common conditions can lead to hyperammonemia in adults, such as fibrolamellar hepatocellular carcinoma. Clinical awareness of hyperammonemic encephalopathy in patients with normal l...
متن کاملHyperammonemic Encephalopathy Associated With Fibrolamellar Hepatocellular Carcinoma: Case Report, Literature Review, and Proposed Treatment Algorithm.
UNLABELLED We report a case of a 31-year-old man with metastatic fibrolamellar hepatocellular carcinoma (FLHCC) treated with gemcitabine and oxaliplatin complicated by hyperammonemic encephalopathy biochemically consistent with acquired ornithine transcarbamylase deficiency. Awareness of FLHCC-associated hyperammonemic encephalopathy and a pathophysiology-based management approach can optimize ...
متن کاملContemporary management of fibrolamellar hepatocellular carcinoma: diagnosis, treatment, outcome, prognostic factors, and recent developments
Fibrolamellar hepatocellular carcinoma (FL-HCC) is a malignant liver tumor which is thought to be a variant of conventional hepatocellular carcinoma (HCC). It accounts for a small proportion of HCC cases and occurs in a distinctly different group of patients which are young and usually not in the setting of chronic liver disease. The diagnosis of FL-HCC requires the integration of clinical info...
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1. Ruffin MT. Fibrolamellar carcinoma. Am J Gastroenterol 1990;85:577...81. 2. Ringe B, Wittekind C, Bechstein WO, et al. The role of liver transplantation in hepatobiliary malignancy. Ann Surg 1989; 209:88-98. 3. Ismail T, Angrisani L, Gunson BK. Primary hepatic malignancy: The role of liver transplantation. Br J Surg 1990;77:983-7. 4. Iwatsuki S, Gordon RD. Role of liver transplantation in ca...
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The role of liver transplantation in the treatment of hepatocellular carcinoma (HCC) is still debated. Overall survival is poor because of the high recurrence rate. Negative prognostic factors are the presence of large, multiple lesions, lymph node involvement, gross vascular invasion and extrahepatic metastases. On the other hand a long term survival (5 yrs), can be achieved in about 30 to 48%...
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