Hepatic regenerating nodules in hereditary tyrosinemia

Hereditary tyrosinemia is an autosomal recessive, enzymatic disorder that results in micro- and macronodular cirrhosis in early childhood. Hepatocellular carcinoma occurs in approximately one-third of affected children. We evaluated the imaging studies performed in five children with this disorder. Pathologic examination of all five of the livers revealed cirrhosis and multiple regenerating nodules; hepatocellular carcinoma was present in two of the five livers. All five patients had high-attenuation or high- and low-attenuation foci within the liver. These high-attenuation foci were not apparent as focal lesions in three of four hepatic sonograms or in one of two hepatic nuclear scans. Angiography showed tumor vascularity in one patient with a focal hepatocellular carcinoma, but was indeterminate in a second patient with severe cirrhosis and multifocal hepatocellular carcinoma. Children with cirrhosis due to tyrosinemia may develop regenerating nodules that appear as high-attenuation hepatic foci on CT scans. It is difficult to differentiate regenerating nodules from multifocal hepatocellular carcinoma in these patients.