Fibrolamellar Carcinoma: A Distinct Variant of Hepatocellular Carcinoma That Is Still Surrounded by Unveils Mysteries

Consolato Sergi^1,2*

¹Department of Laboratory Medicine and Pathology, University of Alberta, Edmonton, Canada.
²Department of Pediatrics, Stollery Children’s Hospital, Edmonton, Canada.

DOI: 10.4236/jct.2014.514132                

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

Conflicts of Interest

The authors declare no conflicts of interest.

References

[1]	Tanaka, Y., Inoue, T. and Horie, H. (2013) International Pediatric Liver Cancer Pathological Classification: Current Trend. International Journal of Clinical Oncology, 18, 946-954. http://dx.doi.org/10.1007/s10147-013-0624-8
[2]	Fabbretti, G., Sergi, C., Consalez, G., Faa, G., Brisigotti, M., Romeo, G. and Callea, F. (1992) Genetic Variants of Alpha-1-Antitrypsin (AAT). Liver, 12, 296-301. Review.
[3]	Torbenson, M. (2007) Review of the Clinicopathologic Features of Fibrolamellar Carcinoma. Advances in Anatomic Pathology, 14, 217-223. Review.
[4]	Klein, W.M., Molmenti, E.P., Colombani, P.M., Grover, D.S., Schwarz, K.B., Boitnott, J. and Torbenson, M.S. (2005) Primary Liver Carcinoma Arising in People Younger than 30 Years. American Journal of Clinical Pathology, 124, 512-518. http://dx.doi.org/10.1309/TT0R7KAL32228E99
[5]	Al-Bahrani, R., Abuetabh, Y., Zeitouni, N. and Sergi, C. (2013) Cholangiocarcinoma: Risk Factors, Environmental Influences and Oncogenesis. Annals of Clinical & Laboratory Science, 43, 195-210.
[6]	Corti, B., D’Errico, A., Pierangeli, F., Fiorentino, M., Altimari, A. and Grigioni, W.F. (2002) Primary Paraganglioma Strictly Confined to the Liver and Mimicking Hepatocellular Carcinoma: An Immunohistochemical and in Situ Hybridization Study. American Journal of Surgical Pathology, 26, 945-949. http://dx.doi.org/10.1097/00000478-200207000-00015
[7]	Cheuk, W. and Chan, J.K. (2001) Clear Cell Variant of Fibrolamellar Carcinoma of the Liver. Archives of Pathology & Laboratory Medicine, 125, 1235-1238.
[8]	Kannangai, R., Vivekanandan, P., Martinez-Murillo, F., Choti, M. and Torbenson, M. (2007) Fibrolamellar Carcinomas Show Over-Expression of Genes in the RAS, MAPK, PIK3, and Xenobiotic Degradation Pathways. Human Pathology, 38, 639-644. http://dx.doi.org/10.1016/j.humpath.2006.07.019
[9]	Bahitham, W., Liao, X., Peng, F., Bamforth, F., Chan, A., Mason, A., Stone, B., Stothard, P. and Sergi, C. (2014) Mitochondriome and Cholangiocellular Carcinoma. PLoS One, 9, e104694. http://dx.doi.org/10.1371/journal.pone.0104694
[10]	Bulterys, M., Goodman, M.T., Smith, M.A., et al. (1999) Hepatic Tumors. In: Ries, L.A.G., Smith, M.A., Gurney, J.G., et al., Eds., Cancer Incidence and Survival among Children and Adolescents: United States SEER Program 1975-1995, SEER Program, NIH Pub. No. 99-4649, National Cancer Institute, Bethesda, MD, 91-98.