Fibrolamellar Carcinoma of the Liver
Hepatocellular carcinoma (HCC) usually develops in cirrhotic livers of older adults. Typically, it is widely disseminated or multicentric at the time of diagnosis, unresectable, and has a poor prognosis with survival time measured in months. Fibrolamellar carcinoma (FLC) is a variant of HCC whose separation as a distinct entity is justified by clinicopathological features which sharply contrast with those commonly observed in HCC (Table 10.1). FLC occurs in children and young adults with noncirrhotic livers and has unique histological features from which its name derives. Edmondson first recognized these characteristics in a case he described in 1956 , and this was followed by the report of five additional cases by Peters in 1976, who referred to them as HCC with lamellar fibrosis . The existence of this neoplasm was subsequently confirmed by two large series in 1980: In the series of Berman et al.  the term HCC of poly-gonal cell type with lamellar fibrosis was used; the other series was by Craig et al. , whose term FLC of the liver has persisted. While this neoplasm constitutes only l%–2% of all HCC , 40% of cases seen in patients under 35 years of age are of this type . At the time of diagnosis, the tumor is frequently localized in the liver, surgically resectable, and potentially curable. The recognition of FLC is consequently of major importance.
KeywordsHepatocellular Carcinoma Focal Nodular Hyperplasia Alpha Fetoprotein Tocellular Carcinoma Endodermal Sinus Tumor
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