Journal of Cancer Therapy, 2014, 5, 1325-1331 Published Online December 2014 in SciRes. http://www.scirp.org/journal/jct http://dx.doi.org/10.4236/jct.2014.514132 How to cite this paper: Sergi, C. (2014) Fibrolamellar Carcinoma: A Distinct Variant of Hepatocellular Carcinoma That Is Still Surrounded by Unveils Mysteries. Journal of Cancer Therapy, 5, 1325-1331. http://dx.doi.org/10.4236/jct.2014.514132 Fibrolamellar Carcinoma: A Distinct Variant of Hepatocellular Carcinoma That Is Still Surrounded by Unveils Mysteries Consolato Sergi 1,2* 1 Department of Laboratory Medicine and Pathology, University of Alberta, Edmonton, Canada 2 Department of Pediatrics, Stollery Children’s Hospital, Edmonton, Canada Email: * sergi@ualberta.ca Received 23 September 2014; revised 20 October 2014; accepted 14 November 2014 Copyright © 2014 by author and Scientific Research Publishing Inc. This work is licensed under the Creative Commons Attribution International License (CC BY). http://creativecommons.org/licenses/by/4.0/ Abstract Fibrolamellar carcinoma (FL-HCC) is overall a quite rare variant of hepatocellular carcinoma (HCC) that is intriguing and poorly understood at same time. In humans, it affects essentially the young population (children and young adults) in the peculiar absence of underlying liver diseases. FL- HCC has distinctive clinico-pathologic features with metastases frequently found in the regional lymph nodes, but also peritoneum and lungs. Although initially considered harboring an indolent course, survival outcomes have been recently updated pointing to a neoplasm concealing many aspects of biogenesis. In this review, morphology, molecular pathways, and prognosis of this rare tumor are presented. Keywords Liver, Tumor, Carcinoma, Pathogenesis, Differential Diagnosis, Therapy, Prognosis 1. Introduction Fibrolamellar carcinoma (FLC/FL-HCC) is a quite rare variant of hepatocellular carcinoma (HCC) and is intri- guing and poorly understood. Children, adolescents and youth are essentially the population affected by this tu- mor. Both sexes are involved and Caucasians are often the ethnics of this population. There is no history of pa- renchymal liver disease. In new pediatric books and literature [1], FL-HCC is reported as a distinct clinical and histologic variant representing almost 1/3 of all HCCs, which are diagnosed at pediatric age. Genetic hemoch- romatosis, tyrosinemia, alpha-1-antitrypsin deficiency as well as progressive familial intrahepatic cholestasis * Corresponding author.