Fibrolamellar Carcinoma: What Is New and Why It Matters

Rondell P. Graham

doi:10.1016/j.path.2018.02.006

Fibrolamellar Carcinoma: What Is New and Why It Matters

Rondell P. Graham

Laboratory Medicine and Pathology

Research output: Contribution to journal › Review article › peer-review

9 Scopus citations

Abstract

Fibrolamellar carcinoma is distinctive at clinical and histologic levels. A novel DNAJB1-PRKACA fusion gene characterizes almost all cases, distinguishes it from other hepatocellular neoplasms, and drives the pathogenesis of this unique tumor. A subset of cases of fibrolamellar carcinoma is associated with alternate mechanisms of protein kinase A activation. This review article discusses common and unusual histologic features of fibrolamellar carcinoma, its differential diagnoses, and how to make the diagnosis while avoiding key pitfalls. The impact of the discovery of the fusion gene on the understanding of the tumor and the prognosis of fibrolamellar carcinoma are also discussed.

Original language	English (US)
Pages (from-to)	377-387
Number of pages	11
Journal	Surgical Pathology Clinics
Volume	11
Issue number	2
DOIs	https://doi.org/10.1016/j.path.2018.02.006
State	Published - Jun 2018

Keywords

Carney complex
Central scar
Fibrolamellar carcinoma
Fibrolamellar hepatocellular carcinoma
Hepatocellular carcinoma
PRKACA
Pale bodies
Protein kinase A

ASJC Scopus subject areas

Surgery
Pathology and Forensic Medicine

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10.1016/j.path.2018.02.006

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title = "Fibrolamellar Carcinoma: What Is New and Why It Matters",

abstract = "Fibrolamellar carcinoma is distinctive at clinical and histologic levels. A novel DNAJB1-PRKACA fusion gene characterizes almost all cases, distinguishes it from other hepatocellular neoplasms, and drives the pathogenesis of this unique tumor. A subset of cases of fibrolamellar carcinoma is associated with alternate mechanisms of protein kinase A activation. This review article discusses common and unusual histologic features of fibrolamellar carcinoma, its differential diagnoses, and how to make the diagnosis while avoiding key pitfalls. The impact of the discovery of the fusion gene on the understanding of the tumor and the prognosis of fibrolamellar carcinoma are also discussed.",

keywords = "Carney complex, Central scar, Fibrolamellar carcinoma, Fibrolamellar hepatocellular carcinoma, Hepatocellular carcinoma, PRKACA, Pale bodies, Protein kinase A",

author = "Graham, {Rondell P.}",

note = "Publisher Copyright: {\textcopyright} 2018 Elsevier Inc.",

year = "2018",

month = jun,

doi = "10.1016/j.path.2018.02.006",

language = "English (US)",

volume = "11",

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TY - JOUR

T1 - Fibrolamellar Carcinoma

T2 - What Is New and Why It Matters

AU - Graham, Rondell P.

PY - 2018/6

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N2 - Fibrolamellar carcinoma is distinctive at clinical and histologic levels. A novel DNAJB1-PRKACA fusion gene characterizes almost all cases, distinguishes it from other hepatocellular neoplasms, and drives the pathogenesis of this unique tumor. A subset of cases of fibrolamellar carcinoma is associated with alternate mechanisms of protein kinase A activation. This review article discusses common and unusual histologic features of fibrolamellar carcinoma, its differential diagnoses, and how to make the diagnosis while avoiding key pitfalls. The impact of the discovery of the fusion gene on the understanding of the tumor and the prognosis of fibrolamellar carcinoma are also discussed.

AB - Fibrolamellar carcinoma is distinctive at clinical and histologic levels. A novel DNAJB1-PRKACA fusion gene characterizes almost all cases, distinguishes it from other hepatocellular neoplasms, and drives the pathogenesis of this unique tumor. A subset of cases of fibrolamellar carcinoma is associated with alternate mechanisms of protein kinase A activation. This review article discusses common and unusual histologic features of fibrolamellar carcinoma, its differential diagnoses, and how to make the diagnosis while avoiding key pitfalls. The impact of the discovery of the fusion gene on the understanding of the tumor and the prognosis of fibrolamellar carcinoma are also discussed.

KW - Carney complex

KW - Central scar

KW - Fibrolamellar carcinoma

KW - Fibrolamellar hepatocellular carcinoma

KW - Hepatocellular carcinoma

KW - PRKACA

KW - Pale bodies

KW - Protein kinase A

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DO - 10.1016/j.path.2018.02.006

M3 - Review article

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AN - SCOPUS:85044134253

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VL - 11

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JO - Surgical Pathology Clinics

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ER -

Fibrolamellar Carcinoma: What Is New and Why It Matters

Abstract

Keywords

ASJC Scopus subject areas

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