Fibrolamellar hepatocellular carcinoma:Exploring molecular mechanisms and differentiation pathways to better understand disease outcomes and prognosis
10.1016/j.livres.2017.12.003
- Author:
A.Kersten CASSANDRA
1
;
N.Sloey ELISE
;
Zhou ERIC
;
Peng YING
;
S.Torbenson MICHAEL
;
Guo YI
Author Information
1. Department of Biochemistry and Molecular Biology
- Keywords:
Fibrolamellar hepatocellular carcinoma(FLC);
DnaJ heat shock protein family member B1(DNAJB1);
Protein kinase cAMP-activated catalytic subunit alpha(PRKACA);
Pediatric cancer;
Liver cancer
- From:
Liver Research
2017;1(4):187-192
- CountryChina
- Language:Chinese
-
Abstract:
Fibrolamellar hepatocellular carcinoma(FLC)is a rare but aggressive liver cancer of children that occurs predominantly in teenagers without a history of liver disease.Surgical resection remains the only therapeutic option,and the recurrence rate is extremely high(＞50％within 3 years).A newly discovered chromosomal deletion that occurs in the majority of FLCs generates a novel kinase fusion between DnaJ heat shock protein family member B1(DNAJB1)and protein kinase cAMP-activated catalytic subunit alpha(PRKACA)(DNAJB1-PRKACA).Despite its high penetrance and apparent specificity for FLC,the oncogenic role of this fusion event remains unclear.In this review article,we discuss the histology,presentation and diagnosis,current treatment,and roles of the DNAJB1-PRKACA as well as research models contributing to our understanding of this disease.
