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Plain English Summary of "Caroli's Syndrome with Incidental Fibrolamellar Carcinoma on Liver Explant"

Published January, 2016 in Indian Journal of Pediatrics

Caroli’s syndrome is a rare and poorly understood inherited disease where the liver bile ducts are abnormally widened and fibrous tissue is present in the liver known as congenital hepatic fibrosis. There is a known increased risk of hepatobiliary cancers but not hepatocellular cancers for this syndrome. In this paper, doctors write about a nine-year-old patient with Caroli’s syndrome who had a successful liver transplant. Upon examination of the diseased liver, doctors found a tumor in the right lobe of the liver and determined it was fibrolamellar hepatocellular carcinoma (FLHCC) based on the pathology of how the hepatocytes and surrounding stroma appeared. This is the first time that FLHCC has been reported in a patient with a previously known liver disease.

-- Melissa Jarmel, Rockefeller University