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Fibrolamellar Hepatocellular Carcinoma: Mechanistic Distinction From Adult Hepatocellular Carcinoma
Author(s) -
Riggle Kevin M.,
Turnham Rigney,
Scott John D.,
Yeung Raymond S.,
Riehle Kimberly J.
Publication year - 2016
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.25970
Subject(s) - hepatocellular carcinoma , pathogenesis , medicine , cancer research , protein subunit , liver disease , mutation , protein kinase a , hccs , disease , gene , kinase , bioinformatics , pathology , biology , genetics
Fibrolamellar hepatocellular carcinoma (FL‐HCC) has historically been classified as a rare subtype of HCC. However, unlike “classic” HCC, it occurs in children and young adults without underlying liver disease. The recent discovery of a deletion mutation in all FL‐HCCs represented a major advancement in understanding the pathogenesis of this disease. This deletion results in the fusion of the genes encoding a heat shock protein ( DNAJB1 ) and the catalytic subunit of protein kinase A (PKA, PRKACA ), and overexpression of PRKACA and enhanced cAMP‐dependent PKA activity. This review summarizes recent advancements in FL‐HCC pathogenesis and characteristics of the HSP40‐PKA C protein.