8171/3 Hepatocellular carcinoma, fibrolamellar


Definitions

Liver and intrahepatic bile ducts
ICD-O-3 topography code: C22
ICD10: C22
           

Fibrolamellar carcinomas (FLC) are distinctive liver cancers of children and young adults that differ from classical hepatocellular carcinoma (HCC) at the clinical, histological, and molecular levels 1
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Torbenson M (2007)
Review of the clinicopathologic features of fibrolamellar carcinoma.
Adv Anat Pathol 14: 217-23



. FLC account for 0.5?9.0% of primary liver cancers in various case series, with reported frequency being influenced by country and study design. Overall, FLC appears to be less common in Asia and in Africa than in North America and European countries. FLC arises in non-cirrhotic livers; etiology and risk factors are not known. There is no strong gender predilection. The age at presentation shows a unimodal distribution peaking at age 25 years and 85% of all FLC occur in individuals aged 35 years or younger. Two-thirds of cases involve the left lobe of the liver. Grossly, FLC tend to be yellow to pale tan and are firm to hard. A central scar may be found in about 75% of cases, but FLC do not appear to be etiologically related to focal nodular hyperplasia. Histologically, FLC typically grow with broad pushing borders and benign portal tracts can occasionally be found entrapped within the growing tumour front. FLC are made up of large polygonal cells with abundant eosinophilic (oncocytic) cytoplasm, large vesicular nuclei, and large nucleoli. These distinctive cytological findings in conjunction with the lamellar fibrosis are the defining features of FLC. The eosinophilic cytoplasm is rich in mitochondria. In a subset of FLC, areas of gland-like formation are present, with tumour cells lining circular or ovoid spaces, some of which may contain mucicarmine and/or alcian blue-positive secretions. Such cases can be misclassified as combined hepatocellular cholangiocarcinoma in the absence of careful attention to the complete histological findings. Other findings include calcifications, either in stroma or as luminal calcification of pseudogland secretions. ?Pale bodies? and/or ?hyaline bodies? may be found, as in classical HCC. The prognosis for FLC is better than for typical HCC that arises in cirrhotic livers, but similar to typical HCC that arises in non-cirrhotic livers2
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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