695 results match your criteria Fibrolamellar Carcinoma


In situ hybridisation for albumin RNA in paediatric liver cancers compared with common immunohistochemical markers.

J Clin Pathol 2020 May 29. Epub 2020 May 29.

Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USA

Aims: In situ hybridisation (ISH) for albumin mRNA is a sensitive marker of primary liver tumours in adults. However, paediatric tumours, such as hepatoblastoma (HB) and fibrolamellar hepatocellular carcinoma (FLC), have not been tested thoroughly and may require ancillary tests to diagnose with confidence. We aim to determine if albumin ISH is useful in the pathological evaluation of these malignancies and to compare it to commonly used immunohistochemical markers HepPar 1 (HEPA) and arginase-1 (ARG). Read More

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http://dx.doi.org/10.1136/jclinpath-2020-206663DOI Listing

A Multicenter Randomized Three-Arm Phase II Study of (1) Everolimus, (2) Estrogen Deprivation Therapy (EDT) with Leuprolide + Letrozole, and (3) Everolimus + EDT in Patients with Unresectable Fibrolamellar Carcinoma.

Oncologist 2020 May 12. Epub 2020 May 12.

Memorial Sloan Kettering Cancer Center, New York, New York, USA.

Lessons Learned: FLC is a complex cancer with many implicated oncogenic pathways. Single or dual targeting does not appear to alter the natural history of the cancer, and novel therapeutics are needed. Estrogen deprivation therapy with letrozole and leuprolide, alone or in combination with the mTOR inhibitor, everolimus, did not demonstrate clinical activity in advanced fibrolamellar carcinoma. Read More

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http://dx.doi.org/10.1634/theoncologist.2020-0367DOI Listing

Clinical features and surgical outcomes of fibrolamellar hepatocellular carcinoma: retrospective analysis of a single-center experience.

World J Surg Oncol 2020 May 12;18(1):93. Epub 2020 May 12.

Department of General, Visceral, and Transplantation Surgery, Ruprecht Karls University Hospital, Im Neuenheimer Feld 110, 69120, Heidelberg, Germany.

Background: Clinicopathological features and surgical outcomes of patients with fibrolamellar hepatocellular carcinoma (FL-HCC) are underreported. The aim of this study is to describe clinical characteristics and surgical outcomes for patients with this rare tumor to raise awareness among clinicians and surgeons.

Methods: Retrospective review of records of a tertiary referral center and specialized liver unit was performed. Read More

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http://dx.doi.org/10.1186/s12957-020-01855-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7218513PMC

Corrigendum to: "BAP1 mutations define a homogeneous subgroup of hepatocellular carcinoma with fibrolamellar-like features and activated PKA" [J Hepatol (2020) 1-13].

J Hepatol 2020 Apr 29. Epub 2020 Apr 29.

Centre de Recherche des Cordeliers, Sorbonne Université, Inserm, Université de Paris, F-75006 Paris, France; Functional Genomics of Solid Tumors laboratory, équipe labellisée Ligue Nationale contre le Cancer, Labex OncoImmunology, F-75006, Paris, France; Hôpital Européen Georges Pompidou, APHP, F-75015 Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.jhep.2020.04.004DOI Listing
April 2020
11.336 Impact Factor

Clinical heterogeneity of pediatric hepatocellular carcinoma.

Pediatr Blood Cancer 2020 Jun 19;67(6):e28307. Epub 2020 Apr 19.

Division of Pediatric Hematology/Oncology, University of Illinois College of Medicine at Peoria, Peoria, Illinois.

Background: Hepatocellular carcinoma (HCC) is often a chemoresistant neoplasm with a poor prognosis. Pediatric HCC may reflect unique biological and clinical heterogeneity.

Procedure: An IRB-approved retrospective institutional review of patients with HCC treated between 2004 and 2015 was undertaken. Read More

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http://dx.doi.org/10.1002/pbc.28307DOI Listing

Hotspots of Aberrant Enhancer Activity in Fibrolamellar Carcinoma Reveal Candidate Oncogenic Pathways and Therapeutic Vulnerabilities.

Cell Rep 2020 Apr;31(2):107509

Department of Biomedical Sciences, College of Veterinary Medicine, Cornell University, Ithaca, NY 14853, USA. Electronic address:

Fibrolamellar carcinoma (FLC) is a rare, therapeutically intractable liver cancer that disproportionately affects youth. Although FLC tumors exhibit a distinct gene expression profile, the chromatin regulatory landscape and the genes most critical for tumor cell survival remain unclear. Here, we use chromatin run-on sequencing to discover ∼7,000 enhancers and 141 enhancer hotspots activated in FLC relative to nonmalignant liver. Read More

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http://dx.doi.org/10.1016/j.celrep.2020.03.073DOI Listing

Population-Based Analysis of Hepatocellular Carcinoma in Children: Identifying Optimal Surgical Treatment.

J Am Coll Surg 2020 Jun 6;230(6):1035-1044.e3. Epub 2020 Apr 6.

Department of Surgery, Division of Hepatobiliary Surgery and Liver Transplantation, Vanderbilt University Medical Center, Nashville, TN. Electronic address:

Background: Hepatocellular carcinoma (HCC) constitutes 0.5% of childhood malignancies and exhibits poor prognosis. Complete tumor extirpation either by partial liver resection (LR) or liver transplantation (LT) is the only curative treatment. Read More

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http://dx.doi.org/10.1016/j.jamcollsurg.2020.03.024DOI Listing

ASO Author Reflections: Fibrolamellar Hepatocellular Carcinoma and Alpha-Fetoprotein.

Ann Surg Oncol 2020 Jun 18;27(6):1906-1907. Epub 2020 Mar 18.

Surgical Oncology Program, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.

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http://dx.doi.org/10.1245/s10434-020-08366-0DOI Listing

Phase II Multicenter, Open-Label Study of Oral ENMD-2076 for the Treatment of Patients with Advanced Fibrolamellar Carcinoma.

Oncologist 2020 Mar 10. Epub 2020 Mar 10.

University of California San Francisco, San Francisco, California, USA.

Lessons Learned: The fibrolamellar carcinoma-associated DNAJB1-PRKACA gene fusion transcript RNA codes for the catalytic domain of protein kinase A and, thus, overexpression of Aurora kinase A. ENMD-2076 showed a favorable toxicity profile. The limited results, one patient (3%) with a partial response and 57% of patients with stable disease, do not support further evaluation of ENMD-2076 as single agent. Read More

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http://dx.doi.org/10.1634/theoncologist.2020-0093DOI Listing
March 2020
4.865 Impact Factor

DnaJ-PKAc fusion induces liver inflammation in a zebrafish model of fibrolamellar carcinoma.

Dis Model Mech 2020 Apr 30;13(4). Epub 2020 Apr 30.

Department of Medical Microbiology and Immunology, University of Wisconsin-Madison, Madison, WI 53706, USA

Fibrolamellar carcinoma (FLC) is a rare liver cancer that affects adolescents and young adults. Genomic analysis of FLC has revealed a 400 kb deletion in chromosome 19 that leads to the chimeric transcript (DnaJ-PKAc), comprised of the first exon of heat shock protein 40 () and exons 2-10 of the catalytic subunit of protein kinase A ). Here, we report a new zebrafish model of FLC induced by ectopic expression of zebrafish Dnaja-Pkaca (zfDnaJa-Pkaca) in hepatocytes that is amenable to live imaging of early innate immune inflammation. Read More

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http://dx.doi.org/10.1242/dmm.042564DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7197716PMC

Imaging Features of Fibrolamellar Hepatocellular Carcinoma with Contrast-Enhanced Ultrasound.

Ultraschall Med 2020 Feb 26. Epub 2020 Feb 26.

Department Allgemeine Innere Medizin, Kliniken Hirslanden Beau Site, Salem und Permanence, Switzerland.

Purpose:  Fibrolamellar hepatocellular carcinoma (f-HCC) is a rare primary liver tumor. Imaging plays an important role in diagnosis. The aim of this retrospective study was to analyze contrast-enhanced ultrasound (CEUS) features of histologically proven f-HCC in comparison to benign focal nodular hyperplasia (FNH). Read More

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http://dx.doi.org/10.1055/a-1110-7124DOI Listing
February 2020
4.924 Impact Factor

Fibrolamellar Carcinoma: Novel Insights into a Rare Subtype.

Ann Surg Oncol 2020 Jun 13;27(6):1733-1734. Epub 2020 Feb 13.

Department of Surgical Oncology, Roger Williams Medical Center, Providence, RI, USA.

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http://dx.doi.org/10.1245/s10434-020-08253-8DOI Listing

Fibrolamellar Hepatocellular Carcinoma: A Population-Based Observational Study.

Dig Dis Sci 2020 Feb 12. Epub 2020 Feb 12.

Division of Gastroenterology and Hepatology, Huntsman Cancer Center, University of Utah School of Medicine, Salt Lake City, UT, USA.

Background: In the USA, fibrolamellar hepatocellular carcinoma (FLC) accounts for 1-2% of all cases of hepatocellular carcinoma. FLC remains poorly understood.

Aim: We aim to investigate the incidence, demographics, tumor characteristics, treatment, and prognosis of patients with FLC. Read More

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http://dx.doi.org/10.1007/s10620-020-06135-3DOI Listing
February 2020

Using dual checkpoint blockade to treat fibrolamellar hepatocellular carcinoma.

Gut 2020 Feb 12. Epub 2020 Feb 12.

Department of Medicine II, University Hospital, LMU Munich, Munich, Germany.

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http://dx.doi.org/10.1136/gutjnl-2020-320604DOI Listing
February 2020

Predictors of Outcome in Patients With Fibrolamellar Carcinoma: Analysis of the National Cancer Database.

Anticancer Res 2020 Feb;40(2):847-855

Department of Medicine, Division of Hematology-Oncology, Stephenson Cancer Center, University of Oklahoma Health Sciences Center, Oklahoma City, OK, U.S.A.

Background: Fibrolamellar carcinoma (FLC) is a very rare liver tumor. We aimed to retrospectively analyze the clinicopathological factors and treatment modalities affecting overall survival (OS) in FLC. The objective of the study was to identify predictors of survival in FLC. Read More

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http://dx.doi.org/10.21873/anticanres.14017DOI Listing
February 2020

Acute Onset Hyperammonemic Encephalopathy Related to Fibrolamellar Carcinoma: Another One Bites the Dust.

Am J Med Sci 2020 04 13;359(4):242-244. Epub 2020 Jan 13.

Hepatology Division, Gastroenteroloy Department, Pró-Fígado-Clínica e Cirurgia das Doenças Hepáticas, São Paulo, São Paulo, Brazil.

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http://dx.doi.org/10.1016/j.amjms.2020.01.004DOI Listing

A classification based on tumor budding and immune score for patients with hepatocellular carcinoma.

Oncoimmunology 2020 7;9(1):1672495. Epub 2019 Nov 7.

Department of Plastic and Burns Surgery, West China Hospital, Sichuan University, Chengdu, China.

: The role of immune profiling and tumor budding in hepatocellular carcinoma (HCC) remains largely unknown. This study evaluated the association between tumor budding and lymphocytic infiltration in HCC. Meanwhile, HCC patients were stratified based on tumor budding grade and immune score. Read More

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http://dx.doi.org/10.1080/2162402X.2019.1672495DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6959452PMC
November 2019
6.283 Impact Factor

State-of-the-art MR Imaging of Uncommon Hepatocellular Tumours: Fibrolamellar Hepatocellular Carcinoma and Combined Hepatocellularcholangiocarcinoma.

Curr Med Imaging Rev 2019 ;15(3):269-280

Department of Radiology, Maria Sklodowska-Curie Memorial Cancer Centre, Institute of Oncology, Warsaw, Poland.

Background: Fibrolamellar Carcinoma (FLC) and Combined Hepatocellular- Cholangiocarcinoma (CHC) are rare primary liver tumours, which are related to different clinical settings. In both tumours, correlation with clinical data and laboratory tests are extremely important.

Discussion: Typically, FLC is diagnosed in young patients without any chronic disease and with normal biochemical tests, whereas CHC arises in cirrhotic patients with elevated tumour markers: AFP and/or CA 19-9. Read More

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http://dx.doi.org/10.2174/1573405614666180927113622DOI Listing
January 2019

Fibrolamellar hepatocellular carcinoma with paraneoplastic neuropsychiatric manifestations.

ANZ J Surg 2020 Jan 22. Epub 2020 Jan 22.

Department of Upper Gastrointestinal Surgery, Bankstown-Lidcombe Hospital, Sydney, New South Wales, Australia.

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http://dx.doi.org/10.1111/ans.15722DOI Listing
January 2020

Elevated Serum α-Fetoprotein is Associated with Abbreviated Survival for Patients with Fibrolamellar Hepatocellular Carcinoma Who Undergo a Curative Resection.

Ann Surg Oncol 2020 Jun 10;27(6):1900-1905. Epub 2020 Jan 10.

Surgical Oncology Program, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.

Background: Fibrolamellar hepatocellular carcinoma (FLC) is a rare variant of hepatocellular carcinoma (HCC), with most clinical data stemming from single-institution series. The variability in the literature lends support for analysis using a large national dataset. In doing so, we sought to (1) define the characteristics and outcomes of patients with FLC; (2) determine factors associated with survival in patients undergoing resection; and (3) compare the overall survival (OS) of patients with FLC with a matched group of patients with HCC. Read More

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http://dx.doi.org/10.1245/s10434-019-08178-xDOI Listing

BAP1 mutations define a homogeneous subgroup of hepatocellular carcinoma with fibrolamellar-like features and activated PKA.

J Hepatol 2020 May 18;72(5):924-936. Epub 2019 Dec 18.

Centre de Recherche des Cordeliers, Sorbonne Université, Inserm, Université de Paris, F-75006 Paris, France; Functional Genomics of Solid Tumors laboratory, équipe labellisée Ligue Nationale contre le Cancer, Labex OncoImmunology, F-75006, Paris, France; Hôpital Européen Georges Pompidou, APHP, F-75015 Paris, France. Electronic address:

Background & Aims: DNAJB1-PRKACA fusion is a specific driver event in fibrolamellar carcinoma (FLC), a rare subtype of hepatocellular carcinoma (HCC) that occurs in adolescents and young adults. In older patients, molecular determinants of HCC with mixed histological features of HCC and FLC (mixed-FLC/HCC) remain to be discovered.

Methods: A series of 151 liver tumors including 126 HCC, 15 FLC, and 10 mixed-FLC/HCC were analyzed by RNAseq and whole-genome- or whole-exome sequencing. Read More

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http://dx.doi.org/10.1016/j.jhep.2019.12.006DOI Listing
May 2020
11.336 Impact Factor

DNAJB1-PRKACA-positive metastatic fibrolamellar carcinoma with unknown primary in a pediatric patient.

Pediatr Blood Cancer 2020 02 17;67(2):e28060. Epub 2019 Nov 17.

Department of Pediatric Hematology and Oncology, Cliniques Universitaires Saint-Luc, Brussels, Belgium.

Fibrolamellar carcinoma (FLC) is a rare variant of hepatocellular carcinoma, occurring in children and young adults without underlying liver disease. The diagnosis is based on morphological characteristics of the tumor, supplemented by immunohistochemistry and/or genetic testing. Recently, the presence of a characteristic DNAJB1-PRKACA fusion gene has been associated with FLC. Read More

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http://dx.doi.org/10.1002/pbc.28060DOI Listing
February 2020

Clavicle tumor as an initial manifestation of fibrolamellar hepatocellular carcinoma.

Rev Gastroenterol Mex 2020 Jan - Mar;85(1):104-106. Epub 2019 Oct 31.

Aparato Digestivo, Hospital García Orcoyen, Estella, España.

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http://dx.doi.org/10.1016/j.rgmx.2019.03.001DOI Listing
October 2019
1 Read

DNAJB1-PRKACA fusions occur in oncocytic pancreatic and biliary neoplasms and are not specific for fibrolamellar hepatocellular carcinoma.

Mod Pathol 2020 04 1;33(4):648-656. Epub 2019 Nov 1.

Memorial Sloan Kettering Cancer Center, New York, NY, USA.

Recently discovered DNAJB1-PRKACA oncogenic fusions have been considered diagnostic for fibrolamellar hepatocellular carcinoma. In this study, we describe six pancreatobiliary neoplasms with PRKACA fusions, five of which harbor the DNAJB1-PRKACA fusion. All neoplasms were subjected to a hybridization capture-based next-generation sequencing assay (MSK-IMPACT), which enables the identification of sequence mutations, copy number alterations, and selected structural rearrangements involving ≥410 genes (n = 6) and/or to a custom targeted, RNA-based panel (MSK-Fusion) that utilizes Archer Anchored Multiplex PCR technology and next-generation sequencing to detect gene fusions in 62 genes (n = 2). Read More

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http://dx.doi.org/10.1038/s41379-019-0398-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7125037PMC
April 2020
8 Reads

Single-Cell RNA Sequencing Identifies Yes-Associated Protein 1-Dependent Hepatic Mesothelial Progenitors in Fibrolamellar Carcinoma.

Am J Pathol 2020 01 24;190(1):93-107. Epub 2019 Oct 24.

Division of Gastroenterology, Department of Medicine, Duke University, Durham, North Carolina. Electronic address:

Fibrolamellar carcinoma (FLC) is characterized by in-frame fusion of DnaJ heat shock protein family (Hsp40) member B1 (DNAJB1) with protein kinase cAMP-activated catalytic subunit α (PRKACA) and by dense desmoplasia. Surgery is the only effective treatment because mechanisms supporting tumor survival are unknown. We used single-cell RNA sequencing to characterize a patient-derived FLC xenograft model and identify therapeutic targets. Read More

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http://dx.doi.org/10.1016/j.ajpath.2019.09.018DOI Listing
January 2020
4 Reads

[Salvage liver transplantation for hepatocellular carcinoma].

Khirurgiia (Mosk) 2019 (10):21-28

State Research Center - Burnasyan Federal Medical Biophysical Center of Federal Medical Biological Agency of Russia, Moscow, Russia.

Objective: To analyze clinical course and the results of salvage liver transplantation in patients with recurrent hepatocellular carcinoma (HCC) after liver resection.

Material And Methods: A 54-year-old man with HCV-infection and HCC and 22-year-old woman with fibrolamellar variant of HCC underwent resection of the right and left liver lobe, respectively. The first patient experienced recurrent HCC four times with an interval of 3-6 months within 2 years after surgery. Read More

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http://dx.doi.org/10.17116/hirurgia201910121DOI Listing
October 2019
1 Read

Hepatocellular Carcinoma of Fibrolamellar Type in an Adolescent: Case Report and Literature Review.

Gastrointest Tumors 2019 Aug 27;6(1-2):43-50. Epub 2019 May 27.

Hospital Regional de Alta Especialidad del Bajío (HRAEB), Servicio de Oncopediatría, Dirección de Planeación, Enseñanza e Investigación, León, Mexico.

We present a female patient, 13 years old, with diagnosis of hepatocellular carcinoma of fibrolamellar type, which was rapidly evolving. The fibrolamellar hepatocellular carcinoma invaded more than 80% of the hepatic parenchyma without surgical possibility or liver transplantation. Measures applied corresponded to chemotherapy of 1 cycle of cisplatin 40 mg/s/5 days + vincristine 1. Read More

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http://dx.doi.org/10.1159/000499581DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6738251PMC
August 2019
1 Read

Detection of Novel Fusion Transcript VTI1A-CFAP46 in Hepatocellular Carcinoma.

Gastrointest Tumors 2019 Aug 10;6(1-2):11-27. Epub 2019 Apr 10.

Division of Gastroenterology and Hepatology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.

Background: Hepatocellular carcinoma (HCC) is now the second-highest cause of cancer death worldwide. Recent studies have discovered a wide range of somatic mutations in HCC. These mutations involve various vital signaling pathways such as: Wnt/-Catenin, p53, telome-rase reverse transcriptase (TERT), chromatin remodeling, RAS/MAPK signaling, and oxidative stress. Read More

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http://dx.doi.org/10.1159/000496795DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6738276PMC
August 2019
2 Reads

Hepatoblastoma and Pediatric Hepatocellular Carcinoma: An Update.

Pediatr Dev Pathol 2020 Mar-Apr;23(2):79-95. Epub 2019 Sep 25.

Department of Pathology, UPMC Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania.

Hepatoblastomas (HBs) and pediatric hepatocellular carcinomas (HCCs) together account for almost 80% of primary malignant liver tumors in children and adolescents/young adults. Children's Hepatic International Collaboration (CHIC), Children's Oncology Group (COG), SociétéInternationale d'Oncologie Pédiatrique (SIOP), and International Childhood Liver Tumors Strategy Group trials have contributed to define prognostic factors and risk stratification in these tumors. The recently proposed histologic International Consensus classification of HB and HCC in children based on retrospective analysis from CHIC cases represents the base to define entities with homogeneous clinicopathologic and molecular features. Read More

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http://dx.doi.org/10.1177/1093526619875228DOI Listing
September 2019
1 Read

A prognostic nomogram for patients with resected fibrolamellar hepatocellular carcinoma.

Hepatobiliary Surg Nutr 2019 Aug;8(4):338-344

Department of Surgery, City of Hope National Medical Center, Duarte, CA, USA.

Background: Fibrolamellar hepatocellular carcinoma (FLHC) is a unique entity compared to conventional hepatocellular carcinoma. The aim of this study was to examine post-resection outcomes and prognostic indicators for survival in this group of FLHC patients.

Methods: A retrospective analysis of the National Cancer Database (NCDB) for patients with FLHC who underwent resection from 2004 to 2014 was performed. Read More

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http://dx.doi.org/10.21037/hbsn.2019.05.03DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6700009PMC
August 2019
10 Reads

Neurotensin as a source of cyclic AMP and co-mitogen in fibrolamellar hepatocellular carcinoma.

Oncotarget 2019 Aug 20;10(49):5092-5102. Epub 2019 Aug 20.

Department of Surgery, University of Washington, Seattle, WA, USA.

Fibrolamellar hepatocellular carcinomas (FL-HCCs) possess a unique mutation that encodes a chimeric form of protein kinase A (DNAJ-PKAc), which includes a chaperonin binding domain. DNAJ-PKAc retains most of the biochemical properties of the native enzyme, however, and activity remains dependent on cAMP. We thus speculated that a persistent source of cAMP is necessary to promote FL-HCC carcinogenesis, and that neurotensin (NTS) may drive cAMP production in this setting, given that NS serum and tumor levels are elevated in many patients with FL-HCC. Read More

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http://dx.doi.org/10.18632/oncotarget.27149DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6707953PMC
August 2019
8 Reads

Emerging Roles of GLI-Similar Krüppel-like Zinc Finger Transcription Factors in Leukemia and Other Cancers.

Authors:
Anton M Jetten

Trends Cancer 2019 09 20;5(9):547-557. Epub 2019 Aug 20.

Cell Biology Section, Immunity, Inflammation and Disease Laboratory, National Institute of Environmental Health Sciences, National Institutes of Health, 111 Alexander Drive, Research Triangle Park, NC 27709, USA. Electronic address:

GLI-similar 1-3 (GLIS1-3), a subfamily of Krüppel-like zinc finger transcription factors, function as key regulators of several biological processes important to oncogenesis, including control of cell proliferation, differentiation, self-renewal, and epithelial-mesenchymal transition. This review provides a short overview of the critical roles genetic changes in GLIS1-3 play in the development of several malignancies. This includes intrachromosomal translocations involving GLIS2 and ETO2/CBFA2T3 in the development of pediatric non-Down's syndrome (DS), acute megakaryoblastic leukemia (AMKL), a malignancy with poor prognosis, and an association of interchromosomal translocations between GLIS3, GLIS1, and PAX8, and between GLIS3 and CLPTM1L with hyalinizing trabecular tumors (HTTs) and fibrolamellar hepatocellular carcinoma (FHCC), respectively. Read More

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http://dx.doi.org/10.1016/j.trecan.2019.07.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6777873PMC
September 2019
6 Reads

Hyperammonemic encephalopathy: An unusual presentation of fibrolamellar hepatocellular carcinoma.

Clin Mol Hepatol 2020 Jan 16;26(1):74-77. Epub 2019 Aug 16.

Department of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA.

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http://dx.doi.org/10.3350/cmh.2018.0042DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6940482PMC
January 2020
3 Reads

Clinical outcomes of rare hepatocellular carcinoma variants compared to pure hepatocellular carcinoma.

J Hepatocell Carcinoma 2019 22;6:119-129. Epub 2019 Jul 22.

Department of Hematology and Medical Oncology, Winship Cancer Institute, Emory University School of Medicine, Atlanta, GA, USA.

Background: HCC variants are rare primary hepatic tumors. The aim of this study is to compare clinical characteristics and outcomes of HCC variants with pure HCC.

Methods: Patients diagnosed between 2004 and 2013 with ICD-O-3 8180/3 and 8170/3-8175/3 were identified from the National Cancer Database. Read More

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http://dx.doi.org/10.2147/JHC.S215235DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6660638PMC
July 2019
4 Reads

MDM4 expression in fibrolamellar hepatocellular carcinoma.

Oncol Rep 2019 Oct 18;42(4):1487-1496. Epub 2019 Jul 18.

Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.

Fibrolamellar hepatocellular carcinoma (FL‑HCC) is a variant of hepatocellular carcinoma (HCC) that most commonly affects adolescents and young adults and is associated with an extremely poor prognosis due to the lack of effective chemotherapeutic agents. Mutations in p53 are a common oncogenic driver in HCC but not in FL‑HCC. However, in tumors lacking a p53 mutation, the tumor suppressor activity of p53 has been revealed to be dysregulated in several different cancer types. Read More

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http://dx.doi.org/10.3892/or.2019.7241DOI Listing
October 2019
7 Reads
2.191 Impact Factor

Progression after Immunotherapy for Fibrolamellar Carcinoma.

Visc Med 2019 Mar 12;35(1):39-42. Epub 2019 Feb 12.

Institute of Pathology, Technische Universität München, Munich, Germany.

Background: Fibrolamellar carcinoma (FLC) is a rare malignancy of the liver that differs from typical hepatocellular carcinoma (HCC) in several aspects such as the absence of underlying liver disease and occurrence in younger patients. Even though the survival rates in FLC are slightly better than in typical HCC, the prognosis of metastatic FLC remains deleterious. Several reports suggest that systemic chemotherapy regimens can successfully be used to halt disease progression in FLC, while targeted tumor therapy with sorafenib seems to be of limited efficiency. Read More

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http://dx.doi.org/10.1159/000497464DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6597907PMC
March 2019
10 Reads

Spontaneous Rupture of Fibrolamellar Variant Hepatocellular Carcinoma.

Gastroenterology Res 2019 Jun 7;12(3):166-170. Epub 2019 Jun 7.

Staten Island University Hospital, Staten Island, NY, USA.

Fibrolamellar hepatocellular carcinoma (FL-HCC) is a unique variant of hepatocellular carcinoma. The majority of cases present with nonspecific symptoms like vague abdominal pain, weight loss and fatigue. Ruptured FL-HCC occurs rarely and mortality in the acute phase is very high. Read More

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http://www.gastrores.org/index.php/Gastrores/article/view/11
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http://dx.doi.org/10.14740/gr1148DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6575133PMC
June 2019
9 Reads

Hyperammonemic encephalopathy in a patient with fibrolamellar hepatocellular carcinoma: case report and literature review.

J Gastrointest Oncol 2019 Jun;10(3):582-588

Department of Medical Oncology, Mayo Clinic, Rochester, MN, USA.

Fibrolamellar hepatocellular carcinoma (fHCC) is a rare primary liver cancer that affects young adults with no prior liver disease. fHCC-associated hyperammonemic encephalopathy (HAE) is an uncommon and life-threatening complication. Hyperammonemia has been reported in both typical and fHCC as a result of intrahepatic shunting, side effect from immunotherapy or chemotherapy, or as a paraneoplastic phenomenon. Read More

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http://dx.doi.org/10.21037/jgo.2019.01.28DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6534716PMC
June 2019
22 Reads

Clinicopathological features and outcomes of fibrolamellar hepatocellular carcinoma.

J Gastrointest Oncol 2019 Jun;10(3):554-561

Department of Medical Oncology, Mayo Clinic, Rochester, MN, USA.

Background: Clinicopathological features and the outcomes of patients with fibrolamellar hepatocellular carcinoma (FLHCC) are not clearly defined.

Methods: Data were collected by retrospective chart review on 42 patients with FLHCC treated between 1990 and 2017 at Mayo Clinic.

Results: Of 42 patients (median age at diagnosis 22 years), 10 patients (23. Read More

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http://jgo.amegroups.com/article/view/27159/20952
Publisher Site
http://dx.doi.org/10.21037/jgo.2019.01.35DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6534717PMC
June 2019
10 Reads

An acquired scaffolding function of the DNAJ-PKAc fusion contributes to oncogenic signaling in fibrolamellar carcinoma.

Elife 2019 05 7;8. Epub 2019 May 7.

Department of Pharmacology, University of Washington Medical Center, Seattle, United States.

Fibrolamellar carcinoma (FLC) is a rare liver cancer. FLCs uniquely produce DNAJ-PKAc, a chimeric enzyme consisting of a chaperonin-binding domain fused to the Cα subunit of protein kinase A. Biochemical analyses of clinical samples reveal that a unique property of this fusion enzyme is the ability to recruit heat shock protein 70 (Hsp70). Read More

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http://dx.doi.org/10.7554/eLife.44187DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6533061PMC
May 2019
9 Reads

Update on hepatocellular carcinoma: Pathologists' review.

World J Gastroenterol 2019 Apr;25(14):1653-1665

Department of Pathology and Laboratory Medicine, Albany Medical College, Albany, NY 12208, United States.

Histopathologic diversity and several distinct histologic subtypes of hepatocellular carcinoma (HCC) are well-recognized. Recent advances in molecular pathology and growing knowledge about the biology associated with distinct histologic features and immuno-profile in HCC allowed pathologists to update classifications. Improving sub-classification will allow for more clinically relevant diagnoses and may allow for stratification into biologically meaningful subgroups. Read More

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http://dx.doi.org/10.3748/wjg.v25.i14.1653DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6465943PMC
April 2019
8 Reads

Road map for fibrolamellar carcinoma: progress and goals of a diversified approach.

J Hepatocell Carcinoma 2019 31;6:41-48. Epub 2019 Jan 31.

Cancer Biology and Genetics Program, Memorial Sloan Kettering Cancer Center, New York, NY, USA,

Fibrolamellar carcinoma is a rare liver cancer, which primarily afflicts adolescents and young adults worldwide and is frequently lethal. Given the rarity of this disease, patient recruitment for clinical trials remains a challenge. In November 2017, the Second Fibrolamellar Cancer Foundation Scientific Summit (Stamford, CT, USA) provided an opportunity for investigators to discuss recent advances in the characterization of the disease and its surrounding liver and immune context. Read More

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http://dx.doi.org/10.2147/JHC.S194764DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6362920PMC
January 2019
13 Reads

Inhibition of the chimeric DnaJ-PKAc enzyme by endogenous inhibitor proteins.

J Cell Biochem 2019 08 2;120(8):13783-13791. Epub 2019 Apr 2.

University of Vermont Cancer Center, Burlington, Vermont.

The chimeric DnaJ-PKAc enzymeresulting from an approximately 400-kb deletion of chromosome 19 is a primary contributor to the oncogenic transformation that occurs in fibrolamellar hepatocellular carcinoma, also called fibrolamellar carcinoma (FLC). This oncogenic deletion juxtaposes exon 1 of the DNAJB1 heat shock protein gene with exon 2 of the PRKACA gene encoding the protein kinase A catalytic subunit, resulting in DnaJ-PKAc fusion under the transcriptional control of the DNAJB1 promoter. The expression of DnaJ-PKAc is approximately 10 times that of wild-type (wt) PKAc catalytic subunits, causing elevated and dysregulated kinase activity that contributes to oncogenic transformation. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1002/jcb.28651
Publisher Site
http://dx.doi.org/10.1002/jcb.28651DOI Listing
August 2019
28 Reads

Structures of the PKA RIα Holoenzyme with the FLHCC Driver J-PKAcα or Wild-Type PKAcα.

Structure 2019 05 21;27(5):816-828.e4. Epub 2019 Mar 21.

Structural Biophysics Laboratory, Center for Cancer Research, National Cancer Institute, Frederick, MD, USA. Electronic address:

Fibrolamellar hepatocellular carcinoma (FLHCC) is driven by J-PKAcα, a kinase fusion chimera of the J domain of DnaJB1 with PKAcα, the catalytic subunit of protein kinase A (PKA). Here we report the crystal structures of the chimeric fusion RIα:J-PKAcα holoenzyme formed by J-PKAcα and the PKA regulatory (R) subunit RIα, and the wild-type (WT) RIα:PKAcα holoenzyme. The chimeric and WT RIα holoenzymes have quaternary structures different from the previously solved WT RIβ and RIIβ holoenzymes. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09692126193007
Publisher Site
http://dx.doi.org/10.1016/j.str.2019.03.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6506387PMC
May 2019
15 Reads

Multiple Immune-Suppressive Mechanisms in Fibrolamellar Carcinoma.

Cancer Immunol Res 2019 05 22;7(5):805-812. Epub 2019 Mar 22.

Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland.

Fibrolamellar carcinoma (FLC) is a rare type of liver cancer that affects adolescents and young adults. The most effective treatment for FLC is surgical resection, but no standardized systemic therapy exists for patients with recurrent or unresectable FLC. As a first step to understand the immune microenvironment of FLC, we investigated targetable immune-checkpoint pathways, PD-1, PD-L1, B7-H3, IDO-1, and LAG3, in relation to CD8 cytotoxic T-lymphocyte density. Read More

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http://dx.doi.org/10.1158/2326-6066.CIR-18-0499DOI Listing
May 2019
13 Reads

Deleted in Liver Cancer 2 (DLC2) protein expression in hepatocellular carcinoma.

Eur J Histochem 2019 Feb 18;63(1). Epub 2019 Feb 18.

Medical University of Warsaw, Department of Pathology.

Deleted in Liver Cancer (DLC) proteins belong to the family of RhoGAPs and are believed to operate as negative regulators of the Rho family of small GTPases. So far, the role of the first identified member from the DLC family, DLC1, was established as a tumor suppressor in hepatocellular carcinoma. The function of its close family relative, DLC2 is unequivocal. Read More

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http://dx.doi.org/10.4081/ejh.2019.2981DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6383041PMC
February 2019
9 Reads