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Semin Liver Dis 2018; 38(01): 051-059
DOI: 10.1055/s-0037-1621710
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Fibrolamellar Carcinoma: Recent Advances and Unresolved Questions on the Molecular Mechanisms

Gadi Lalazar
1   The Laboratory for Cellular Biophysics, The Rockefeller University, New York, New York
,
Sanford M. Simon
1   The Laboratory for Cellular Biophysics, The Rockefeller University, New York, New York
› Author Affiliations
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Publication History

Publication Date:
22 February 2018 (online)

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Abstract

Fibrolamellar hepatocellular carcinoma (FLC) is a rare form of primary liver cancer that affects adolescents and young adults without underlying liver disease. Surgery remains the mainstay of therapy; however, most patients are either not surgical candidates or suffer from recurrence. There is no approved systemic therapy and the overall survival remains poor. Historically classified as a subtype of hepatocellular carcinoma (HCC), FLC has a unique clinical, histological, and molecular presentation. At the genomic level, FLC contains a single 400kB deletion in chromosome 19, leading to a functional DNAJB1-PRKACA fusion protein. In this review, we detail the recent advances in our understanding of the molecular underpinnings of FLC and outline the current knowledge gaps.

Keywords

liver cancer - pediatric cancer - adolescent young adult cancer - fusion protein - protein kinase A