Two case reports of childhood liver cell adenomas harboring beta-catenin abnormalities.
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.
Department of Pediatric Surgery, Tokyo University, Tokyo, Japan.
Hajime
H
Takayasu
Toru
T
Motoi
Yutaka
Y
Kanamori
Yoshihiro
Y
Kitano
Hiroko
H
Nakanishi
Tsuyoshi
T
Tange
Akira
A
Nakagawara
Kohei
K
Hashizume
D018248:Adenoma, Liver Cell / Q000737:chemistry* / Q000235:genetics / Q000473:pathology; D000293:Adolescent; D001483:Base Sequence; D002467:Cell Nucleus / Q000737:chemistry; D002648:Child; D003598:Cytoskeletal Proteins / Q000032:analysis* / Q000235:genetics; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000737:chemistry* / Q000235:genetics / Q000473:pathology; D009154:Mutation; D016133:Polymerase Chain Reaction; D015534:Trans-Activators / Q000032:analysis* / Q000235:genetics; D051176:beta Catenin
C495270:CTNNB1 protein, human; D003598:Cytoskeletal Proteins; D015534:Trans-Activators; D051176:beta Catenin