Fibrolamellar hepatocarcinoma: clinical, radiologic, and pathologic features

Three new cases of an unusual subtype of hepatocellular carcinoma (HCC) referred to as fibrolamellar hepatocarcinoma (FLHC) recently seen at our institution are described. This report focuses on the clinical, radiologic, and pathologic features of this rare subset of HCC. All three patients were under 30 years of age with no previous history of hepatitis or cirrhosis. Each had had subacute symptoms for 5 months to 1 year before medical attention was sought and/or diagnosis of FLHC was established. There was no reliable correlation with oral contraceptive use in the 2 female patients. Serum alpha-1-fetoprotein levels were normal with only mild elevation of liver enzymes. The CT features, although not specific, were suggestive of an aggressive tumor with amorphous calcification in 2 of the 3 cases. Angiographically all tumors were hypervascular without any evidence of arterioportal shunting or venous invasion of major vessels. The clinical and radiologic recognition of these tumors is important since the surgical resectibility rate and 2- and 5-year survival rates are higher than those applicable to conventional HCC.