Hum Pathol 1988 Jul;19(7):784-94

Fibrolamellar carcinoma of the liver: an immunohistochemical study of ninetee
cases and a review of the literature

Berman MA, Burnham JA, Sheahan DG
Department of Pathology, Presbyterian-University Hospital, Pittsburgh, PA 15213.

Abstract
Hepatocellular carcinoma (HCC) is a rapidly fatal neoplasm of high worldwide
prevalence. Fibromellar carcinoma (FLC), a variant of HCC, lacks the dismal
prognosis of "ordinary" HCC (O-HCC) and is characterized by a diagnostic
histologic appearance. The current study analyzes the clinical characteristics,
immunohistochemistry, and treatment of nineteen cases of FLC. These data,
together with a detailed review of the literature, further characterize this
unique variant. FLC affects younger patients and lacks the male predominance of
O-HCC. Also, FLC lacks specific association with cirrhosis, hepatitis B virus
infection, use of oral contraceptives, and alcohol abuse, all of which are
implicated in other hepatic tumors. This, along with differences in serum tumor
marker prevalence (AFP, B12 binding protein) suggests that its pathogenesis
differs from that of O-HCC. Despite these differences, FLC shares a common
differentiation with O-HCC. The increased amounts in FLC of stainable
alpha-1-antitrypsin, fibrinogen, and C-reactive protein, all of which are acute
phase reactants and normal hepatocyte products, implies better differentiation
of FLC cells. Finally, the better prognosis of FLC is supported by this study,
since only two of the 19 patients died because of tumor. This contrasts with the
reported survival of patients with O-HCC, usually measured in weeks. Hepatic
transplantation may hold promise for future patients with "surgically
unresectable" FLC as procedure-related complications are overcome