Gastroenterology

Gastroenterology

Volume 128, Issue 5, May 2005, Pages 1211-1218
Gastroenterology

Clinical-liver, pancreas, and biliary tract
Clinical, Morphologic, and Molecular Features Defining So-Called Telangiectatic Focal Nodular Hyperplasias of the Liver

https://doi.org/10.1053/j.gastro.2005.02.004Get rights and content

Background & Aims: 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 1α (HNF1α) 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%). HNF1α 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 HNF1α mutations and require a similar type of treatment. However, morphologic and molecular data support the hypothesis that TFNH is a separate entity.

Section snippets

Patients

Between July 1992 and July 2003, we saw 13 patients at the University Hospital of Bordeaux who had a hepatic resection for TFNH. For all cases included in this study, full clinical data and follow-up were available. As comparison groups, we studied FNH and adenoma/adenomatosis exhibiting typical pathologic features, at least in a part of the tumor, leading to a firm diagnosis. For the first control group, we used 28 FNHs that were collected between 1998 and June 2003. For the second, we used 17

Pathologic Features of TFNH

In our series, the diagnosis of TFNH was straightforward in 8 cases (Table 1). In these cases, 6 features were constantly present: (1) soft tumor, (2) partially preserved lobulation with maintained acinar structures, (3) centrolobular/mediolobular sinusoidal dilatation from moderate to severe (peliosis), diffuse or occupying large areas, (4) presence of portal tract-like structures containing one or several dystrophic arteries (usually less than in FNH), with (5) an inflammatory infiltrate and

Discussion

In the present series, the diagnosis of TFNH was based on pathologic examination. We used 6 criteria to define TFNH, which allowed us to individualize TFNH from adenoma and FNH. The strongest argument to suggest the diagnosis of TFNH, present in 8 cases, was the general appearance of lobulation with the presence of portal tract-like structures containing one or several arteries with a thickened wall and an inflammatory lymphocytic infiltrate, the sinusoidal dilatation, and the ductular

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    Supported by the Association pour la recherche sur le Cancer (ARC no. 3108), the INSERM (Réseaux de recherche clinique et réseaux de recherche en santé des populations), the comité Dordogne de la Ligue contre le Cancer, and the SNFGE. S.R. is supported by a Ligue Nationale Contre le Cancer doctoral fellowship.

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