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PLoS One 2018 21;13(11):e0206791. Epub 2018 Nov 21.

Paleontological Institute and Museum, University of Zurich, Zurich, Switzerland.

The abundance of skeletal remains of cave bears in Pleistocene deposits can offer crucial information on the biology and life history of this megafaunal element. The histological study of 62 femora from 23 different European localities and comparisons with specimens of five extant ursid species revealed novel data on tissue types and growth patterns. Cave bear's femoral bone microstructure is characterized by a fibrolamellar complex with increasing amounts of parallel-fibered and lamellar bone towards the outer cortex. Read More

Chin Clin Oncol 2018 Oct;7(5):51

GI & HPB Service, Department of Surgical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India.

Fibrolamellar hepatocellular carcinoma (FLHCC) is a primary liver tumor. It is a pathologically distinct variety of hepatocellular carcinoma (HCC). The term 'fibrolamellar' is derived from the presence of thick fibrous collagen bands surrounding the tumor cells. Read More

Anat Rec (Hoboken) 2018 Sep;301(9):1506-1518

Museo Paleontológico Egidio Feruglio, Av. Fontana 140, Trelew, Chubut Province, U9100GYO, Argentina.

This analysis of the long bone microstructure of Antetonitrus ingenipes fills a crucial gap in our understanding of the growth dynamics of sauropodomorph dinosaurs. The bone histology of basal Sauropodomorpha are often characterized by zonal tissue, and contrasts with that of more derived sauropod taxa which show a shift toward the deposition of uninterrupted fibrolamellar bone (with lines of growth being either absent or only present in the outer circumferential layer). In Antetonitrus, growth patterns in the youngest individuals exhibit uninterrupted fibrolamellar bone without any growth marks. Read More

AME Case Rep 2018 10;2:21. Epub 2018 May 10.

Department of Surgical Gastroenterology, Rajiv Gandhi Government General Hospital & Madras Medical College, Chennai 600003, India.

Primary hepatic lymphoma (PHL) is a lymphoma presenting with predominant liver involvement at presentation in the early stage of lymphoma. It accounts to less than 0.4% of all extranodal lymphomas and usually occurs in immunocompromised states. Read More

Pediatr Blood Cancer 2018 Sep 26:e27475. Epub 2018 Sep 26.

Tytgat Institute for Liver and Intestinal Research, Amsterdam, The Netherlands.

Pediatric hepatocellular carcinoma (HCC) is rare, resulting in scattered knowledge of tumor biology and molecular background. Thus far, the variant in children has been treated as a different entity from adult HCC. We weigh the hypothesis that HCC in the pediatric and adult groups may be the same entity and may benefit from the same treatment. Read More

Arch Pathol Lab Med 2018 Sep;142(9):1141-1145

From the Department of Pathology and Cell Biology, Columbia University Medical Center, New York, New York. Dr Hui-Min Yang is currently located in the Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, British Columbia, Canada.

Fibrolamellar carcinoma is a rare primary hepatocellular malignancy arising in noncirrhotic livers of young individuals. Patients commonly present with a large solitary liver mass and nonspecific symptoms. Characteristic histologic features include large polygonal cells with oncocytic cytoplasm and prominent nucleoli separated into trabeculae and cords by dense parallel bands of collagen. Read More

Ann Transl Med 2018 Jul;6(14):285

Northwest Liver Research Program, Department of Surgery, University of Washington, Seattle, Washington, USA.

With the widespread adoption of molecular profiling in clinical oncology practice, many physicians are faced with making therapeutic decisions based upon isolated genomic alterations. For example, epidermal growth factor receptor tyrosine kinase inhibitors (TKIs) are effective in EGFR-mutant non-small cell lung cancers (NSCLC) while anti-EGFR monoclonal antibodies are ineffective in Ras-mutant colorectal cancers. The matching of mutations with drugs aimed at their respective gene products represents the current state of "precision" oncology. Read More

Intern Med 2018 Aug 10. Epub 2018 Aug 10.

Department of Gastroenterology, Aichi Cancer Center Hospital, Japan.

A 20-year old man was diagnosed with fibrolamellar hepatocellular carcinoma (FLHCC) with multiple lung metastases, and chemotherapy with FOLFOX was administered. Contrast enhanced CT after 3 cycles of FOLFOX showed no disease progression. We therefore performed surgical resection and radiofrequency ablation of the liver lesions and lung metastases, after obtaining the patient's informed consent. Read More

Pediatr Blood Cancer 2018 Dec 9;65(12):e27392. Epub 2018 Aug 9.

Division of Pediatric Oncology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Ornithine transcarbamylase deficiency (OTCD) disrupts the metabolic pathway responsible for converting nitrogenous waste to urea, allowing for excretion. When impaired, ammonia levels accumulate in the blood resulting in severe, sometimes life-threatening toxicities. Abnormalities of the urea cycle are often inherited, though there are some rarer acquired forms. Read More

Sci Rep 2018 Jul 30;8(1):9945. Epub 2018 Jul 30.

Université de Toulouse, IRD, UPS, UMR 152 PHARMADEV, Toulouse, France.

We previously described a divergent clinical and molecular presentation of hepatocellular carcinoma (HCC) in Peru. The present study aimed to further characterize the tissue features associated with this singular nosological form of HCC in order to gain insight into the natural history of the disease. We performed an exploratory analysis of the histology of both tumor and non-tumor liver (NTL) tissues from 50 Peruvian HCC patients, and compared with that of 75 individuals with non-HCC liver tumor or benign liver lesions as a baseline for NTL features. Read More

Pediatr Blood Cancer 2018 Nov 3;65(11):e27293. Epub 2018 Jul 3.

Division of Oncology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Background: Hepatocellular carcinoma (HCC) is an aggressive malignant neoplasm that is often chemoresistant. Complete surgical resection remains the mainstay of therapy. The role of liver transplantation (LT) in pediatric HCC is in evolution, as is the role of adjuvant chemotherapy for stage I disease. Read More

Abdom Radiol (NY) 2018 Dec;43(12):3340-3348

Division of Diagnostic Imaging, Body Imaging Section, Unit 1473, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX, 77030-4009, USA.

Objective: The aim of our study is to describe the multiphasic CT features of fibrolamellar hepatocellular carcinomas (FLHCCs) and pattern of distant metastases.

Materials And Methods: 33 patients (mean age 28 years: 17M/16F) with pathologically confirmed FLHCC and pre-treatment multiphasic CT were included in the study. Two abdominal radiologists evaluated the multiphasic CT imaging features of these 33 FLHCC patients in consensus. Read More

Gastroenterology 2018 10 5;155(4):e9-e10. Epub 2018 Jun 5.

Division of Hepatobiliary and Pancreas Surgery, Department of Surgery, Mayo Clinic, Rochester, Minnesota.

Surg Pathol Clin 2018 Jun 21;11(2):377-387. Epub 2018 Mar 21.

Division of Anatomic Pathology, Mayo Clinic, 200 First Street Southwest, Rochester, MN 55905, USA; Division of Laboratory Genetics and Genomics, Mayo Clinic, 200 First Street Southwest, Rochester, MN 55905, USA. Electronic address:

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. Read More

Surg Pathol Clin 2018 Jun 26;11(2):367-375. Epub 2018 Mar 26.

Department of Pathology and Cell Biology, Columbia University, 622 W 168th Street, Vanderbilt Clinic 14-209, New York, NY 10032, USA. Electronic address:

Tissue diagnosis is the gold standard for mass lesions of the liver, but needle core biopsies may sometimes prove challenging. Presented here is a review of a panel of immunohistochemical stains, including hepatocyte in paraffin 1, arginase-1, polyclonal carcinoembryonic antigen, CD10, bile salt export pump, glypican-3, as well as in situ hybridization for albumin RNA, to establish hepatocellular origin in cases in which hepatocellular carcinoma is suspected but the sample is limited or the morphology is challenging, as it may be with cases of scirrhous, fibrolamellar carcinoma, intrahepatic cholangiocarcinoma, and combined hepatocellular-cholangiocarcinoma. Read More

J Cytol 2018 Apr-Jun;35(2):75-78

Department of Cytology and Gynecological Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Objective: To distinguish fibrolamellar hepatocellular carcinoma (FL-HCC) variant from the conventional hepatocellular carcinoma (HCC) by cytology, immunocytochemistry, and morphometry.

Study Design: Retrospective detailed cytomorphological, immunocytochemical, and morphometric analysis was performed in 6 cases of FL-HCC reported on fine needle aspiration. Cell block immunocytochemistry (CB-ICC) for CK7 and CD68 was performed in four cases. Read More

Exp Clin Transplant 2018 Apr 9. Epub 2018 Apr 9.

From the Department of General Surgery, Liver Transplantation Institute, Inonu University, Malatya, Turkey.

Fibrolamellar hepatocellular carcinoma is a rare primary malignant liver neoplasm. Benefits from liver transplant for patients with fibrolamellar hepatocellular carcinoma have not yet been reported. Here, we report a 19-year-old female patient who presented with abdominal pain. Read More

Acta Gastroenterol Belg 2017 Jul-Sep;80(3):422-424

Service de Gastro-entérologie, CHU Tivoli (ULB), La Louvière, Belgium.

A 34-year-old woman in the 22nd week of gestation presented with generalized pruritis and weight loss since the first trimester of pregnancy. Physical examination revealed cutaneous scratch lesions, jaundice, and hepatomegaly. Blood tests revealed cholestasis with elevated direct bilirubinemia. Read More

Oncotarget 2018 Feb 15;9(12):10211-10227. Epub 2017 Dec 15.

Laboratory of Cellular Biophysics, The Rockefeller University, New York, 10065 NY, USA.

Fibrolamellar hepatocellular carcinoma (FLC) is a rare primary liver cancer found in adolescents and young adults without underlying liver disease. A deletion of ~400 kD has been found in one copy of chromosome 19 in the tumor tissue of all patients tested. This produces a fusion of the genes DNAJB1 and PRKACA which, in turn, produces a chimeric transcript and protein. Read More

Med J Malaysia 2018 Feb;73(1):46-48

Sarawak General Hospital, Department of Radiotherapy and Oncology, Sarawak, Malaysia.

Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare variant of hepatocellular carcinoma. It is commonly reported in the younger population with no underlying chronic liver disease and free of viral Hepatitis B and C. Local recurrence and distant metastasis are common despite better prognosis compared to conventional hepatocellular carcinoma. Read More

Clin Gastroenterol Hepatol 2018 Mar 2. Epub 2018 Mar 2.

Gastrointestinal Unit, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Cancer Imaging 2018 Mar 1;18(1). Epub 2018 Mar 1.

Department of Diagnostic and Interventional Radiology, Heidelberg University Hospital, INF 110, 69120, Heidelberg, Germany.

Background: Fibrolamellar hepatocellular carcinoma (FLC) is a rare malignancy occurring in young patients without cirrhosis. Objectives of our study were to analyze contrast material uptake in hepatobiliary phase imaging (HBP) in gadoxetic acid-enhanced liver MRI in patients with FLC and to characterize imaging features in sequence techniques other than HBP.

Methods: In this retrospective study on histology-proven FLC, contrast material uptake in HBP was quantitatively assessed by calculating the corrected FLC enhancement index (CEI) using mean signal intensities of FLC and lumbar muscle on pre-contrast imaging and HBP, respectively. Read More

Hepatology 2018 Jul 14;68(1):384-386. Epub 2018 May 14.

Department of Internal Medicine, Spital Emmental, Burgdorf, Switzerland.

Semin Liver Dis 2018 02 22;38(1):51-59. Epub 2018 Feb 22.

The Laboratory for Cellular Biophysics, The Rockefeller University, New York, New York.

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. Read More

Cardiovasc Intervent Radiol 2018 May 21;41(5):816-820. Epub 2018 Feb 21.

Department of Interventional Radiology, Freeman Hospital, Newcastle upon Tyne, NE7 7DN, UK.

We describe a 52-year-old female patient who presented with a 9.5-cm fibrolamellar hepatocellular carcinoma (FL-HCC). The patient was initially unsuitable for surgical resection and therefore underwent transarterial chemoembolization followed by selective internal radiation therapy (SIRT) with Yttrium-90 to downsize the tumour. Read More

Sci Rep 2018 01 15;8(1):720. Epub 2018 Jan 15.

Laboratory of Cellular Biophysics, The Rockefeller University, 1230 York Avenue, New York, NY, 10065, USA.

In fibrolamellar hepatocellular carcinoma a single genetic deletion results in the fusion of the first exon of the heat shock protein 40, DNAJB1, which encodes the J domain, with exons 2-10 of the catalytic subunit of protein kinase A, PRKACA. This produces an enzymatically active chimeric protein J-PKAcα. We used molecular dynamics simulations and NMR to analyze the conformational landscape of native and chimeric kinase, and found an ensemble of conformations. Read More

Pediatr Blood Cancer 2018 Apr 29;65(4). Epub 2017 Dec 29.

Laboratory of Cellular Biophysics, The Rockefeller University, New York City, New York.

Fibrolamellar hepatocellular carcinoma (FLHCC) is a rare liver malignancy in adolescents and young adults. Surgery is the mainstay of therapy for primary and metastatic disease. Most patients relapse, with development of both local and distant metastases. Read More

Hepatology 2018 Oct 11;68(4):1441-1447. Epub 2018 May 11.

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.

Fibrolamellar carcinomas are characterized by activation of protein kinase A, a kinase composed of catalytic and regulatory subunits. PRKACA encodes a catalytic subunit of protein kinase A, and almost all fibrolamellar carcinomas have a heterozygous 400-kb deletion that leads to the fusion of DNAJB1 and PRKACA. The resulting DNAJB1-PRKACA fusion transcript is believed to activate protein kinase A by dysregulation of the catalytic portion of the protein. Read More

J Pathol 2018 Mar 2;244(3):257-259. Epub 2018 Jan 2.

Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), NIH and Section on Endocrinology and Genetics (SEGEN), NICHD, NIH, Bethesda, USA.

Protein kinase A (PKA) or cyclic-AMP (cAMP)-dependent kinase was among the first serine-threonine kinases to be molecularly and functionally characterized. For years, it was investigated as the enzyme that mediates cAMP functions in almost all cell systems and organisms studied to date. Despite PKA's critical role in signaling and the long history of investigations of cAMP in oncogenesis (dating back to the 1970s), it was not until relatively recently that PKA defects were found to be directly involved in tumor predisposition. Read More

Anat Rec (Hoboken) 2018 Jun 1;301(6):1026-1045. Epub 2018 Feb 1.

Department of Geology and Mineralogy, Graduate School of Science, Kyoto University, Sakyoku Kitashirakawa Oiwakecho, Kyoto, 606-8502, Japan.

Despite its importance in various disciplines, a general method to assess ontogenetic ages of skeletal and fossil specimens has been lacking for birds. Although the textural ageing method was formulated to assess relative ontogenetic ages of specimens from inspection of bone surface textures, the exact correspondence of surface textures to ontogenetic stages has not yet been clear. In this study, bone surface textures of six major limb bones (humerus, ulna, carpometacarpus, femur, tibiotarsus, and tarsometatarsus) were described in postnatal ontogenies of four species of modern birds (Calonectris leucomelas, Phalacrocorax capillatus, Larus crassirostris, and Cerorhinca monocerata) from 14 to 28 individuals of known ontogenetic stages for each species. Read More

Proc Natl Acad Sci U S A 2017 12 21;114(50):13076-13084. Epub 2017 Nov 21.

Department of Cancer Biology and Genetics, Memorial Sloan Kettering Cancer Center, New York, NY 10065;

A segmental deletion resulting in gene fusion is now recognized as the signature genetic event of fibrolamellar hepatocellular carcinoma (FL-HCC), a rare but lethal liver cancer that primarily affects adolescents and young adults. Here we implement CRISPR-Cas9 genome editing and transposon-mediated somatic gene transfer to demonstrate that expression of either the endogenous fusion protein or a chimeric cDNA leads to the formation of indolent liver tumors in mice that closely resemble human FL-HCC. Notably, overexpression of the wild-type PRKACA was unable to fully recapitulate the oncogenic activity of , implying that FL-HCC does not simply result from enhanced PRKACA expression. Read More

Eur Radiol 2018 Apr 27;28(4):1529-1539. Epub 2017 Oct 27.

Department of Radiology, University of North Carolina at Chapel Hill, 101 Manning Drive, Chapel Hill, NC, 27514, USA.

Purpose: To determine if rare primary malignancies of the liver may have consistent features on magnetic resonance imaging (MRI).

Materials And Methods: This IRB-compliant retrospective study reviewed the records from the pathology departments of four university centres over an 11-year period from 2005-2016 to identify rare primary malignant tumours, which were cross-referenced with MRI records. MRI studies of these patients were reviewed to determine if these tumours exhibited consistent and distinctive features. Read More

J Comp Pathol 2017 Aug - Oct;157(2-3):80-84. Epub 2017 Jul 23.

Department of Veterinary Services, Wildlife Reserve Singapore, Singapore.

A 38-year-old Malayan gharial (Tomistoma schlegelii) with a 2-week history of anorexia was found dead and presented for post-mortem examination. Numerous white firm nodules of various sizes were found on the surface of the liver, both left and right kidneys, the spleen and the serosa of the intestinal tract. All masses had similar microscopical appearance and were diagnosed as metastasizing fibrolamellar hepatocellular carcinoma. Read More

Gastroenterology 2017 12 18;153(6):1662-1673.e10. Epub 2017 Sep 18.

Biotech Research and Innovation Centre, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark. Electronic address:

Background & Aims: Fibrolamellar hepatocellular carcinoma (FL-HCC) is a primary liver cancer that predominantly affects children and young adults with no underlying liver disease. A somatic, 400 Kb deletion on chromosome 19 that fuses part of the DnaJ heat shock protein family (Hsp40) member B1 gene (DNAJB1) to the protein kinase cAMP-activated catalytic subunit alpha gene (PRKACA) has been repeatedly identified in patients with FL-HCC. However, the DNAJB1-PRKACA gene fusion has not been shown to induce liver tumorigenesis. Read More

Mod Pathol 2018 01 1;31(1):141-149. Epub 2017 Sep 1.

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.

Fibrolamellar carcinoma has a distinctive morphology and immunophenotype, including cytokeratin 7 and CD68 co-expression. Despite the distinct findings, accurate diagnosis of fibrolamellar carcinoma continues to be a challenge. Recently, fibrolamellar carcinomas were found to harbor a characteristic somatic gene fusion, DNAJB1-PRKACA. Read More

Ann Clin Lab Sci 2017 Aug;47(4):395-402

Department of Pediatrics, Stanford University School of Medicine, Stanford, CA, USA

Objectives: CD47 is a transmembrane receptor that inhibits phagocytosis. Over-expression of CD47 is associated with an increased risk of tumor growth and metastasis. Clinical trials based on anti-CD47 therapy in adults are underway in a variety of malignancies. Read More

Pan Afr Med J 2017 16;27:41. Epub 2017 May 16.

Université de Tunis El Manar, Faculté de Médecine de Tunis, 1007.

Hepatocellular carcinoma (HCC) is the most common of all liver cancers and is a major worldwide public health problem. The aim of this study was to provide an updated overview on clinicopathological features, treatment and outcome of HCC. In our retrospective study, we reviewed 64 cases of HCC that were diagnosed at the pathology department of Mongi Slim hospital over a fifteen-year period (2000- 2014). Read More

Acta Radiol 2018 Apr 31;59(4):393-401. Epub 2017 Jul 31.

1 Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.

Background Central scars are rarely reported in conventional hepatocellular carcinoma (HCC). The presence of central scars on imaging might lead to erroneous diagnosis of hepatic tumors. Purpose To determine imaging features of HCC with central scars on magnetic resonance imaging (MRI) including gadoxetic acid-enhanced and diffusion-weighted imaging (DWI). Read More

Pediatr Blood Cancer 2017 Dec 23;64(12). Epub 2017 Jun 23.

Department of Pediatrics, Mattel Children's Hospital at UCLA, Los Angeles, California.

Although checkpoint inhibitor therapies have demonstrated significant efficacy in many malignancies, they have not been well studied in patients with a history of solid organ transplant. We describe two patients with recurrent, refractory, and progressive advanced fibrolamellar hepatocellular carcinoma (HCC) following orthotopic liver transplantation who received programmed cell death protein 1 (PD-1) inhibitor, nivolumab, on a patient access, off-label basis. Both rapidly developed irreversible acute liver rejection shortly after starting therapy, and ultimately died. Read More

Int J Surg Case Rep 2017 7;37:26-32. Epub 2017 Jun 7.

Department of Surgery, Division of Hepatobiliary and Pancreatic Surgery, University of Pittsburgh Medical Center, PA, USA. Electronic address:

Introduction: Mixed hepatocellular and cholangiocarcinoma tumors (MHCC) are described in the literature, as are the more rare mixed adenoneuroendocrine carcinomas (MANC) of hepatobiliary origin. Only two cases of tumors with characteristics of all three histologies/phenotypes have been previously described in one Chinese study.

Presentation Of Case: Herein we report clinical, microscopic and molecular features of a 25cm mixed hepatic tumor with hepatocellular, cholangiocarcinoma and neuroendocrine differentiation arising in an otherwise healthy 19-year-old North American Caucasian male without any identifiable risk factors. Read More

J Clin Gastroenterol 2018 Mar;52(3):262-267

Department of Radiology and Biomedical Imaging, Yale School of Medicine, Division of Interventional Radiology.

Background: Primary liver cancer, including Hepatoblastoma (HB) and hepatocellular carcinoma (HCC), in pediatric populations is often fatal. The outcomes are poor despite universal health care access in pediatric patients.

Aim: We investigated the sociodemographic factors affecting outcomes in pediatric patients with primary liver cancer. Read More

Int J Surg Case Rep 2017 12;36:50-54. Epub 2017 May 12.

Department of Surgery, Western Michigan University Homer Stryker School of Medicine/West Michigan Cancer Center, Kalamazoo, MI, United States. Electronic address:

Background: Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare and unique variant of hepatocellular carcinoma (HCC) whose presentation remains inadequately described. We present a resectable case of FL-HCC which involved tumor thrombus of the common bile duct.

Presentation: A 27 year-old male presenting with jaundice, abdominal pain, vomiting, hepatic dysfunction and hyperbilirubinemia was found to have a large liver mass and lymphadenopathy on preoperative imaging. Read More

Rom J Morphol Embryol 2017 ;58(1):187-192

Center of General Surgery and Liver Transplantation, "Fundeni" Clinical Institute, Bucharest, Romania;

Aim: Fibrolamellar carcinoma (FLC) has been considered a distinct clinical entity vs. hepatocellular carcinoma, with respect to its epidemiology, etiology, and prognosis.

Case Presentation: We describe the unusual case of a 23-year-old female patient with FLC and ovarian (Krukenberg) and peritoneal metastases, clinically mimicking an ovarian carcinoma. Read More

Gastroenterol Clin North Am 2017 06;46(2):365-391

Department of Laboratory Medicine and Pathology, Mayo Clinic Rochester, Rochester, MN, USA. Electronic address:

Hepatocellular carcinomas can be further divided into distinct subtypes that provide important clinical information and biological insights. These subtypes are distinct from growth patterns and are on based on morphologic and molecular findings. There are 12 reasonably well-defined subtypes as well as 6 provisional subtypes, together making up 35% of all hepatocellular carcinomas. Read More

PLoS One 2017 9;12(5):e0176562. Epub 2017 May 9.

Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York, United States of America.

Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare variant of HCC that most frequently affects young adults. Because of its rarity and an absence of preclinical models, our understanding of FL-HCC is limited. Our objective was to analyze chromosomal alterations and dysregulated gene expression in tumor specimens collected at a single center during two decades of experience with FL-HCC. Read More

Fam Cancer 2018 01;17(1):119-122

Genetics Division, Instituto Nacional de Câncer, Rio de Janeiro, Brazil.

Li-Fraumeni syndrome is a rare hereditary cancer predisposition syndrome associated with germline pathogenic variants in TP53 gene. The phenotype may vary from classical to variant forms, known as Li-Fraumeni-like phenotypes. We searched for pathogenic variants in TP53 in a 14 year-old female diagnosed with fibrolamellar hepatocellular carcinoma, a rare subtype of hepatocellular carcinoma. Read More

Histopathology 2017 Aug 22;71(2):217-226. Epub 2017 May 22.

Department of Pathology, Brain Korea 21 PLUS Project for Medical Science, Integrated Genomic Research Center for Metabolic Regulation, Yonsei University College of Medicine, Seoul, Korea.

Aims: The scirrhous variant of hepatocellular carcinoma (S-HCC) and fibrolamellar HCC (FL-HCC) are less common subtypes of HCC that are characterized by abundant fibrous stroma. Here, we aimed to investigate differences in the tumour microenvironment and the tumour epithelial cell characteristics of S-HCC and FL-HCC.

Methods And Results: Whole tissue sections of 17 S-HCCs and 9 FL-HCCs were subjected to immunohistochemical stains for keratin 7 (K7), K19, EpCAM, CD56/NCAM, CD163, CD68, pSTAT3, FAP, CCN2 and Ki-67. Read More

Ann Hepatobiliary Pancreat Surg 2017 Feb 28;21(1):48-51. Epub 2017 Feb 28.

Department of HPB and Liver Transplantation, PSG Institute of Medical Sciences and Research, Avinashi Road, Coimbatore, India.

The Fibrolamellar variant of Hepatocellular Carcinoma (FLHCC) is a rare form of liver cancer that presents in the 3 decade of life, is rarely associated with cirrhosis or chronic Hepatitis B/C virus infection, and usually presents with normal serum alpha-fetoprotein (AFP) levels. FLHCC presenting during pregnancy is extremely rare, with only 4 cases reported. We present a case of FLHCC in pregnancy and discuss the dilemmas in management. Read More

Sci Rep 2017 03 17;7:44653. Epub 2017 Mar 17.

Curriculum in Genetics and Molecular Biology, Chapel Hill, NC, USA.

Fibrolamellar carcinoma (FLC) is a unique liver cancer primarily affecting young adults and characterized by a fusion event between DNAJB1 and PRKACA. By analyzing RNA-sequencing data from The Cancer Genome Atlas (TCGA) for >9,100 tumors across ~30 cancer types, we show that the DNAJB1-PRKACA fusion is specific to FLCs. We demonstrate that FLC tumors (n = 6) exhibit distinct messenger RNA (mRNA) and long intergenic non-coding RNA (lincRNA) profiles compared to hepatocellular carcinoma (n = 263) and cholangiocarcinoma (n = 36), the two most common liver cancers. Read More

World J Gastrointest Surg 2017 Feb;9(2):61-67

Mohamed Abdel Wahab, Ehab El Hanafy, Ayman El Nakeeb, Mahmoud Abdelwahab Ali, Gastroenterology Surgical Center, Mansoura University, Mansoura 35516, Egypt.

Aim: To evaluate the clinicopathological features and the surgical outcomes of patients with fibrolamellar hepatocellular carcinoma (FL-HCC) over a 15-year period.

Methods: This is a retrospective study including 22 patients with a pathologic diagnosis of FL-HCC who underwent hepatectomy over a 15-year period. Tumor characteristics, survival and recurrence were evaluated. Read More