Fibrolamellar Carcinoma: Recent Advances and Unresolved Questions on the Molecular Mechanisms

被引：52

作者：

Lalazar, Gadi ^{[1
]}

Simon, Sanford M. ^{[1
]}

机构：

[1] Rockefeller Univ, Lab Cellular Biophys, New York, NY 10065 USA

来源：

SEMINARS IN LIVER DISEASE | 2018年 / 38卷 / 01期

关键词：

liver cancer; pediatric cancer; adolescent young adult cancer; fusion protein; protein kinase A; PROTEIN-KINASE-A; ADRENAL CUSHINGS-SYNDROME; BETA-CATENIN GENE; HEPATOCELLULAR-CARCINOMA; CATALYTIC SUBUNIT; NATIONAL PERSPECTIVE; SOMATIC MUTATIONS; BREAST-CANCER; AURORA-A; LIVER;

D O I：

10.1055/s-0037-1621710

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

100201 [内科学];

摘要：

Fibrolamellar hepatocellular carcinoma (FLC) is a rare form of primary liver cancer that affects adolescents and young adults without underlying liver disease. Surgery remains the mainstay of therapy; however, most patients are either not surgical candidates or suffer from recurrence. There is no approved systemic therapy and the overall survival remains poor. Historically classified as a subtype of hepatocellular carcinoma (HCC), FLC has a unique clinical, histological, and molecular presentation. At the genomic level, FLC contains a single 400kB deletion in chromosome 19, leading to a functional DNAJB1-PRKACA fusion protein. In this review, we detail the recent advances in our understanding of the molecular underpinnings of FLC and outline the current knowledge gaps.

引用

页码：51 / 59

页数：9

共 72 条

[1]

Fibrolamellar Hepatocellular Carcinoma: An Immunohistochemical Comparison With Conventional Hepatocellular Carcinoma [J].

Abdul-Al, Hala M. ;

Wang, Guanghua ;

Makhlouf, Hala R. ;

Goodman, Zachary D. .

INTERNATIONAL JOURNAL OF SURGICAL PATHOLOGY, 2010, 18 (05) :313-318

[2]

Randomized phase II study of everolimus (E), leuprolide plus letrozole (LL), and E plus LL (ELL) in patients (pts) with unresectable fibrolamellar carcinoma (FLC) [J].

Abou-Alfa, Ghassan K. ;

Mayer, Robert J. ;

Cosgrove, David ;

Capanu, Marinela ;

Choti, Michael A. ;

Atreya, Chloe Evelyn ;

Ang, Celina ;

Kelley, Robin Kate ;

Do, Richard Kinh Gian ;

Gordan, John Dozier ;

Zhu, Andrew X. ;

Ly, Michele ;

Nolan, Patrick ;

Lubin, Lakeisha ;

Harding, James J. ;

Saltz, Leonard ;

Venook, Alan P. .

JOURNAL OF CLINICAL ONCOLOGY, 2015, 33 (15)

[3]

Allosteric inhibitors of Bcr-abl-dependent cell proliferation [J].

Adrián, FJ ;

Ding, Q ;

Sim, TB ;

Velentza, A ;

Sloan, C ;

Liu, Y ;

Zhang, GB ;

Hur, W ;

Ding, S ;

Manley, P ;

Mestan, J ;

Fabbro, D ;

Gray, NS .

NATURE CHEMICAL BIOLOGY, 2006, 2 (02) :95-102

[4]

Role of the ras-association domain family 1 tumor suppressor gene in human cancers [J].

Agathanggelou, A ;

Cooper, WN ;

Latif, F .

CANCER RESEARCH, 2005, 65 (09) :3497-3508

[5]

Ang Celina S, 2013, Gastrointest Cancer Res, V6, P3

[6]

Liver Transplantation for Fibrolamellar Hepatocellular Carcinoma: A National Perspective [J].

Atienza, Leonardo Garcia ;

Berger, Jonathan ;

Mei, Xiaonan ;

Shah, Malay B. ;

Daily, Michael F. ;

Grigorian, Alla ;

Gedaly, Roberto .

JOURNAL OF SURGICAL ONCOLOGY, 2017, 115 (03) :319-323

[7]

Constitutive Activation of PKA Catalytic Subunit in Adrenal Cushing's Syndrome [J].

Beuschlein, Felix ;

Fassnacht, Martin ;

Assie, Guillaume ;

Calebiro, Davide ;

Stratakis, Constantine A. ;

Osswald, Andrea ;

Ronchi, Cristina L. ;

Wieland, Thomas ;

Sbiera, Silviu ;

Faucz, Fabio R. ;

Schaak, Katrin ;

Schmittfull, Anett ;

Schwarzmayr, Thomas ;

Barreau, Olivia ;

Vezzosi, Delphine ;

Rizk-Rabin, Marthe ;

Zabel, Ulrike ;

Szarek, Eva ;

Salpea, Paraskevi ;

Forlino, Antonella ;

Vetro, Annalisa ;

Zuffardi, Orsetta ;

Kisker, Caroline ;

Diener, Susanne ;

Meitinger, Thomas ;

Lohse, Martin J. ;

Reincke, Martin ;

Bertherat, Jerome ;

Strom, Tim M. ;

Allolio, Bruno .

NEW ENGLAND JOURNAL OF MEDICINE, 2014, 370 (11) :1019-1028

[8]

A homologue of Drosophila aurora kinase is oncogenic and amplified in human colorectal cancers [J].

Bischoff, JR ;

Anderson, L ;

Zhu, YF ;

Mossie, K ;

Ng, L ;

Souza, B ;

Schryver, B ;

Flanagan, P ;

Clairvoyant, F ;

Ginther, C ;

Chan, CSM ;

Novotny, M ;

Slamon, DJ ;

Plowman, GD .

EMBO JOURNAL, 1998, 17 (11) :3052-3065

[9]

Activating hotspot L205R mutation in PRKACA and adrenal Cushing's syndrome [J].

Cao, Yanan ;

He, Minghui ;

Gao, Zhibo ;

Peng, Ying ;

Li, Yanli ;

Li, Lin ;

Zhou, Weiwei ;

Li, Xiangchun ;

Zhong, Xu ;

Lei, Yiming ;

Su, Tingwei ;

Wang, Hang ;

Jiang, Yiran ;

Yang, Lin ;

Wei, Wei ;

Yang, Xu ;

Jiang, Xiuli ;

Liu, Li ;

He, Juan ;

Ye, Junna ;

Wei, Qing ;

Li, Yingrui ;

Wang, Weiqing ;

Wang, Jun ;

Ning, Guang .

SCIENCE, 2014, 344 (6186) :913-917

[10]

Unique Phenotype of Hepatocellular Cancers with Exon-3 Mutations in Beta-Catenin Gene [J].

Cieply, Benjamin ;

Zeng, Gang ;

Proverbs-Singh, Tracy ;

Geller, David A. ;

Monga, Satdarshan P. S. .

HEPATOLOGY, 2009, 49 (03) :821-831

← 1 2 3 4 5 6 7 8 →