Two case reports of childhood liver cell adenomas harboring β-catenin abnormalities

Department of Pediatric Surgery, Tokyo University, Tokyo, Japan.
Human Pathlogy (Impact Factor: 2.77). 09/2002; 33(8):852-5. DOI: 10.1053/hupa.2002.125771
Source: PubMed


The benign epithelial neoplasm liver cell adenoma is rare, especially in childhood. We report 2 such cases, 1 of which was associated with Prader-Willi syndrome. Differential diagnosis of the liver cell adenomas on the basis of histopathologic findings proved difficult and was based on the absence of cellular and nuclear atypia, mitotic activity, and invasive growth. In both cases, immunohistochemical staining demonstrated the nuclear accumulation of beta-catenin, and in 1 case, the tumor cells carried a mutation of the beta-catenin gene. Recently, disregulation of the Wnt/beta-catenin pathway, attributable to abnormalities of the beta-catenin gene, has been reported to be a major event in the development of hepatocellular carcinomas and hepatoblastomas. Our report may be the first to describe the beta-catenin abnormalities in childhood liver cell adenoma. These findings imply that abnormalities of beta-catenin can be an early initiating event in human liver tumorigenesis.

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    • "for INK4-ARF inactivation and microdeletion of p14 ARF and p16 INK4a INK4a-ARF (CDKN2A) locus on chromosome 9p21 encodes the tumour suppressor proteins p14 ARF and p16 INK4a and is frequently inactivated in many human cancers Alterations of p14 ARF and p16 INK4a in 3/25 and 6/25 liver adenomas, respectively, not necessarily associated with malignancy Takayasu H, 2002 [87] "
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    ABSTRACT: During the last three decades liver cell adenoma and liver cell adenomatosis have emerged as new clinical entities in hepato-logical practice due to the widespread use of oral contraceptives and increased imaging of the liver. On review of published series there is evidence that 10% of liver cell adenomas progress to hepatocellular carcinoma, diagnosis is best made by open or laparoscopic excision biopsy, and the preferred treatment modality is resection of the liver cell adenoma to prevent bleeding and malignant transformation. In liver cell adenomatosis, the association with oral contraceptive use is not as high as in solitary liver cell adenomas. The risk of malignant transformation is not increased compared with solitary liver cell adenomas. Treatment consists of close monitoring and imaging, resection of superficially located, large (>4 cm) or growing liver cell adenomas. Liver transplantation is the last resort in case of substantive concern about malignant transformation or for large, painful adenomas in liver cell adenomatosis after treatment attempts by liver resection.
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