Adenoma of the Liver

Wilens, Gustav

doi:10.1001/archpedi.1938.01980100128009

Cited by 5 publications

(2 citation statements)

References 2 publications

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“…It is believed to occur in females only, starting in puberty. There are, however, reports of proven cases of hepatic adenoma in girls and in very young boys in infancy or tod-dler age [6,13,25,33]. Consequently, there will be very few patients with FNH who have to undergo exploratory surgery for the definite diagnosis -those without uptake of colloid sulfur and, angiographically, without the typical septa and central scar.…”

Section: Discussionmentioning

confidence: 99%

Focal nodular hyperplasia of the liver imaging by differing modalities

et al. 1983

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The logistic approach in diagnosis of focal nodular hyperplasia (FNH) of the liver is discussed, based on the experience with three children. In only one child could the diagnosis be made without angiography. In that child the combination of hypervascularity on the radionuclide angiogram and uptake of the radiocolloid by Kupffer cells was sufficient for the diagnosis of FNH. If the radionuclide scintigraphy is inconclusive, angiography has to be done to show the typical features of FNH, seen in all three patients. Only if scintigraphy and angiography are not able to differentiate FNH from hepatic adenoma, biopsy or exploratory laparotomy is indicated as a final diagnostic procedure. Ultrasonography or computerized tomography is of value only in screening for hepatic mass lesions.

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Section: Discussionmentioning

confidence: 99%

Focal nodular hyperplasia of the liver imaging by differing modalities

et al. 1983

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“…13 In the latter study the most common subtype identified was IHCA (35%) followed by H-HCA (29%), b-HCA (16%), b-IHCA (10%) and U-HCA (10%). 13 In infants HCA are exceedingly rare highlighted by the fact that only 5 cases, to the best of our knowledge have been reported in the literature, one in a 2 days old boy, 11 a 3 weeks old boy, 14 a 2 months old boy, 11 a 8 months old boy 15 and a 10 months old girl. 12 Interestingly, in the study by Pacheco et al, 13 in 9 of 10 prepubertal children with HCA an underlying syndrome was identified.…”

Section: Introductionmentioning

confidence: 99%

Hepatocellular Adenoma in an Infant With Burn-McKeown Syndrome: Report of a Case

2022

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Hepatocellular adenomas (HCA) in infants are exceedingly rare with only 5 cases reported to the best of our knowledge, all of them preceding the classification of HCA. Here we present an autopsy case of a 9-month-old girl with Burn-McKeown syndrome with an incidental liver nodule in the right lobe measuring 1.5 cm in greatest dimension. The lesion was composed of an unencapsulated proliferation of hepatocytes with multiple unaccompanied arteries without well-formed portal tracts, and an intact reticulin framework without thickened hepatic plates, findings consistent with an HCA. Glutamine synthetase (GS), lipid fatty acid-binding protein (LFABP), c-reactive protein (CRP), serum amyloid-a (SAA), beta-catenin and CD34 immunostains were performed. GS was diffusely and strongly positive in the lesion, CD34 showed heterogenous staining of sinusoids within the lesion without a well-formed rim from the background liver and beta-catenin was negative for nuclear staining. CRP and SAA were considered negative, and LFABP was retained. Molecular testing showed no CTNNB1 variants and found two tier 3 variants involving CHEK2 and PTEN genes. These findings are consistent with an unclassified HCA (U-HCA) per the 2019 WHO Classification of Tumors, representing the youngest patient reported. This raises the possibility that some HCAs are congenital or develop very early in life, remaining undiagnosed until later in life.

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