Telangiectatic Focal Nodular Hyperplasia of the Liver in the Perinatal Period: Case Report

Abstract

We report a case of congenital telangiectatic focal nodular hyperplasia, a rare variant form of the disease. The patient was a 2-month-old boy whose parents noticed abdominal distention about 2 weeks after birth, and ultrasonogram revealed a large mass in the liver. He underwent right lobectomy, and gross findings showed an ill-defined mass without any central scar. Histologic findings demonstrated proliferating hepatocytes without atypia arranged in cords of 1- or 2-cell thickness with marked sinusoidal dilatation and extramedullary hematopoiesis. In addition, a significantly increased Ki-67 labeling index in the tumor compared with non-tumor liver cells, and cytogenetic analysis of 23 G-banded metaphase preparations revealed 3 abnormal karyotypes, suggesting hyperplastic or neoplastic features. To the best of our knowledge, the present case is only the third documented case of congenital telangiectatic focal nodular hyperplasia.

Study of an Ovarian Sclerosing Stromal Tumor Presenting as Vaginal Bleeding in a 7-Month-Old

“…Yet this does not have to be the case, as even monoclonality is neither necessary nor sufficient to characterize a proliferation of cells as being neoplastic in the classical sense of the term. Chromosomal abnormalities have been reported in multinodular thyroid goiters [10] and focal nodular hyperplasia of the liver [11]. The high MIB-1 proliferative index, demonstrated in the epithelioid component of this lesion, is also compatible with the concept of nodular hyperplasia, having been demonstrated in thyroid hyperplastic lesions, nodular goiters, and Graves' disease [12].…”

Retraction: The Effect of Raw and Processed Common Vetch Seed (<i>Vicia sativa</i>) Added to Diets of Laying Hens on Performance, Egg Quality, Blood Parameters and Liver Histopathology

“…Whereas, vascular disorders and another hepatic lesions revealed by effect of raw vetch seed. It is reported that increased arterial flow leads to sinusoidal dilatations (Okamura et al, 2005). Irregular sinusoidal structures also give signs of congestions (Ozturk et al, 2005).…”

Calcified Telangiectatic Hyperplastic Nodule Associated With Vascular Malformation in a Child: A Case Report

“…TFNH can present with clinical hemorrhage, abdominal pain, abnormal liver function tests (mainly increases in gamma glutamyltranspeptidase or alkaline phosphatase) or as an incidental discovery during the performance of a radiological procedure. In all previously reported pediatric cases, TFNH clinically presented with abdominal mass but without jaundice or cholestasis [7][8][9]. Also, there are some cases related to neoplasms, vascular malformations and liver diseases like primary biliary cirrhosis and chronic hepatitis B.…”

“…In children, the scarce descriptions of TFNH report common features like the lack of FNH typical central scar at imaging and the presence of enlarged and proliferating vessels with strong expression of CD34 in the telangiectatic sinusoids at histopathologic examination [7][8][9]. Very little data is available about the pathogenesis of TFNH but the use of molecular techniques has shown that they are monoclonal lesions similar to HA although they do not carry HNF1α mutations [10].…”

“…According to literature, TFNH tend to bleed more frequently than HA [8], so surgical treatment has been advocated because of the risk of hemorrhage in case of multiple or large lesions but also of the risk of developing HCC in adult patients. In pediatric cases, one case of FNH detected at birth spontaneously regressed [9], but in all other published cases, the size of the lesion and the uncertainty of lesion's malignancy was reason to perform surgery [7,8].…”