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Fibrolamellar Hepatocellular Carcinoma: Mechanistic Distinction From Adult Hepatocellular Carcinoma #MMPMID26990031
Riggle KM; Turnham R; Scott JD; Yeung RS; Riehle KJ
Pediatr Blood Cancer 2016[Jul]; 63 (7): 1163-7 PMID26990031show ga
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.