Fibrolamellar hepatocellular carcinoma is a rare subtype of hepatocellular carcinoma with distinct clinical and histological features. The tumor is typically associated with young age, absence of cirrhosis, normal alpha fetoprotein levels and absence of risk factors. Studies suggest that the prognosis of fibrolamellar carcinomas may be better than conventional HCC arising in cirrhosis, but similar to HCC arising in a noncirrhotic liver.
Therefore, this diagnosis should be entertained in young patients, particularly female, who present with a well-circumscribed, malignant appearing lesion, and no known risk factors for chronic liver disease. Liver biopsy is important to make the diagnosis.
The typical histological features of fibrolamellar carcinoma: large cells with eosinophilic granular cytoplasm, prominent nucleoli and a lamellar pattern of fibrosis. This unique pattern of fibrosis is well represented in the pathology specimen above.