title: Clinical, morphologic, and molecular features defining so-called telangiectatic focal nodular hyperplasias of the liver.
abstract: OBJECTIVE
  Telangiectatic focal nodular hyperplasia (TFNH) of the liver is generally believed to belong to the focal nodular hyperplasia (FNH) family. The aim of this study was to use molecular markers, in addition to morphologic features, to better characterize TFNH.
  
  
  METHODS
  Thirteen patients with TFNH were compared with 28 patients with FNH and 17 patients with hepatocellular adenoma. Full clinical and morphologic data were analyzed. Molecular markers included determination of clonality by examining the active X chromosome, genome-wide allelotyping, a search for hepatocyte nuclear factor 1alpha (HNF1alpha) mutations, and determination of ANGPT1/ANGPT2 transcript levels.
  
  
  RESULTS
  No clinical differences were evident between patients with TFNH and adenoma; in particular, bleeding was observed in 77% and 53% of the cases, respectively. Patients with TFNH were more likely to experience nodule recurrence and the presence of multiple nodules than those with either FNH or adenoma. All TFNH and adenoma samples that were available for analysis were monoclonal, in contrast to 40% of the FNH samples. Chromosome losses confirmed monoclonality and were significantly less frequent in TFNH and FNH (22% and 26%) than in adenoma (53%). HNF1alpha mutations were found exclusively in half of the adenomas. ANGPT2 was overexpressed in TFNH and down-regulated in adenoma (P < .01) and FNH (P < .0005).
  
  
  CONCLUSIONS
  TFNHs are monoclonal lesions frequently subject to bleeding that are similar to adenomas not carrying HNF1alpha mutations and require a similar type of treatment. However, morphologic and molecular data support the hypothesis that TFNH is a separate entity.
authors.affiliation: Department of Pathology, Centre Hospitalier Universitaire de Bordeaux, France.
authors.forename: Paulette
authors.initials: P
authors.lastname: Bioulac-Sage
authors.forename: Sandra
authors.initials: S
authors.lastname: Rebouissou
authors.forename: Antonio
authors.initials: A
authors.lastname: Sa Cunha
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Jeannot
authors.forename: Sébastien
authors.initials: S
authors.lastname: Lepreux
authors.forename: Jean-Frédéric
authors.initials: JF
authors.lastname: Blanc
authors.forename: Hélène
authors.initials: H
authors.lastname: Blanché
authors.forename: Brigitte
authors.initials: B
authors.lastname: Le Bail
authors.forename: Jean
authors.initials: J
authors.lastname: Saric
authors.forename: Pierre
authors.initials: P
authors.lastname: Laurent-Puig
authors.forename: Charles
authors.initials: C
authors.lastname: Balabaud
authors.forename: Jessica
authors.initials: J
authors.lastname: Zucman-Rossi
mesh_terms: D018248:Adenoma, Liver Cell / Q000098:blood supply / Q000235:genetics / Q000473:pathology; D000328:Adult; D042683:Angiopoietin-1 / Q000235:genetics; D000091302:Angiopoietin-Like Protein 2; D000075802:Angiopoietin-like Proteins; D042682:Angiopoietins; D015415:Biomarkers; D001798:Blood Proteins / Q000235:genetics; D041321:Chromosomes, Human, X; D004268:DNA-Binding Proteins / Q000235:genetics; D005260:Female; D020518:Focal Nodular Hyperplasia / Q000235:genetics* / Q000473:pathology*; D015894:Genome, Human; D051537:Hepatocyte Nuclear Factor 1; D051538:Hepatocyte Nuclear Factor 1-alpha; D006801:Humans; D036341:Intercellular Signaling Peptides and Proteins; D008113:Liver Neoplasms / Q000098:blood supply / Q000235:genetics* / Q000473:pathology*; D008875:Middle Aged; D009154:Mutation; D009687:Nuclear Proteins / Q000235:genetics; D012333:RNA, Messenger / Q000032:analysis; D013684:Telangiectasis / Q000235:genetics* / Q000473:pathology*; D014157:Transcription Factors / Q000235:genetics
chemical_list: C467630:ANGPT1 protein, human; C485792:ANGPTL2 protein, human; D042683:Angiopoietin-1; D000091302:Angiopoietin-Like Protein 2; D000075802:Angiopoietin-like Proteins; D042682:Angiopoietins; D015415:Biomarkers; D001798:Blood Proteins; D004268:DNA-Binding Proteins; C496487:HNF1A protein, human; D051538:Hepatocyte Nuclear Factor 1-alpha; D036341:Intercellular Signaling Peptides and Proteins; D009687:Nuclear Proteins; D012333:RNA, Messenger; D014157:Transcription Factors; D051537:Hepatocyte Nuclear Factor 1