Fibrolamellar hepatocellular carcinoma: Exploring molecular mechanisms and differentiation pathways to better understand disease outcomes and prognosis

Cassandra A. Kersten, Elise N. Sloey, Eric Zhou, Ying Peng, Michael S. Torbenson, Yi Guo

Research output: Contribution to journalReview article

1 Citation (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)187-192
Number of pages6
JournalLiver Research
Volume1
Issue number4
DOIs
StatePublished - Dec 2017

Fingerprint

Protein Kinases
Catalytic Domain
HSP40 Heat-Shock Proteins
Penetrance
Liver Neoplasms
Heat-Shock Proteins
Liver Diseases
Histology
Phosphotransferases
Recurrence
Therapeutics
Research
Fibrolamellar hepatocellular carcinoma

Keywords

  • DnaJ heat shock protein family member B1 (DNAJB1)
  • Fibrolamellar hepatocellular carcinoma (FLC)
  • Liver cancer
  • Pediatric cancer
  • Protein kinase cAMP-activated catalytic subunit alpha (PRKACA)

ASJC Scopus subject areas

  • Gastroenterology
  • Hepatology

Cite this

Fibrolamellar hepatocellular carcinoma : Exploring molecular mechanisms and differentiation pathways to better understand disease outcomes and prognosis. / Kersten, Cassandra A.; Sloey, Elise N.; Zhou, Eric; Peng, Ying; Torbenson, Michael S.; Guo, Yi.

In: Liver Research, Vol. 1, No. 4, 12.2017, p. 187-192.

Research output: Contribution to journalReview article

@article{f3d2c7009e2c439f873dfcdc0e310c4c,
title = "Fibrolamellar hepatocellular carcinoma: Exploring molecular mechanisms and differentiation pathways to better understand disease outcomes and prognosis",
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.",
keywords = "DnaJ heat shock protein family member B1 (DNAJB1), Fibrolamellar hepatocellular carcinoma (FLC), Liver cancer, Pediatric cancer, Protein kinase cAMP-activated catalytic subunit alpha (PRKACA)",
author = "Kersten, {Cassandra A.} and Sloey, {Elise N.} and Eric Zhou and Ying Peng and Torbenson, {Michael S.} and Yi Guo",
year = "2017",
month = "12",
doi = "10.1016/j.livres.2017.12.003",
language = "English (US)",
volume = "1",
pages = "187--192",
journal = "Liver Research",
issn = "2542-5684",
number = "4",

}

TY - JOUR

T1 - Fibrolamellar hepatocellular carcinoma

T2 - Exploring molecular mechanisms and differentiation pathways to better understand disease outcomes and prognosis

AU - Kersten, Cassandra A.

AU - Sloey, Elise N.

AU - Zhou, Eric

AU - Peng, Ying

AU - Torbenson, Michael S.

AU - Guo, Yi

PY - 2017/12

Y1 - 2017/12

N2 - 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.

AB - 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.

KW - DnaJ heat shock protein family member B1 (DNAJB1)

KW - Fibrolamellar hepatocellular carcinoma (FLC)

KW - Liver cancer

KW - Pediatric cancer

KW - Protein kinase cAMP-activated catalytic subunit alpha (PRKACA)

UR - http://www.scopus.com/inward/record.url?scp=85073625399&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85073625399&partnerID=8YFLogxK

U2 - 10.1016/j.livres.2017.12.003

DO - 10.1016/j.livres.2017.12.003

M3 - Review article

AN - SCOPUS:85073625399

VL - 1

SP - 187

EP - 192

JO - Liver Research

JF - Liver Research

SN - 2542-5684

IS - 4

ER -