title: Hepatocyte nuclear factor-1 α inactivated hepatocellular adenomas in patient with congenital absence of the portal vein: a case report.
abstract: Hepatocellular adenomas (HCAs) have been recognized recently as a heterogeneous group, and are subclassified according to genotype as well as morphological characteristics. We report a case of a 35-year-old Japanese woman who exhibited hepatocyte nuclear factor (HNF)-1α-inactivated HCA in the background of the congenital absence of the portal vein (CAPV). On a dynamic contrast computed tomography (CT) scan, the hypovascular tumor enlarged from 1 cm to 3 cm and another tumor emerged in the course of 7 years. Because the possibility of hepatocellular carcinoma (HCC) with multiple metastases was not excluded, partial hepatectomy was performed. On a cut section, two well-demarcated tumors were observed and one tumor had a central fibrous scar. The histological features of these tumors were similar to those of focal nodular hyperplasia (FNH) with a central scar and HCA; however, these tumors were diagnosed as HNF-1α-inactivated HCA by immunohistochemistry according to the criteria of the current World Health Organization (WHO) classification. In non-tumorous liver tissue, an abnormal architecture of the vessels and a vague nodular appearance of lobuli were observed, which were likely to be those of nodular regenerated hyperplasia (NRH). We discuss its pathogenesis and relationship with CAPV.
authors.affiliation: Department of Pathology, Yokohama City University Graduate School of Medicine, Kanazawa-ku, Yokohama, Japan. ytateish@yokohama-cu.ac.jp
authors.forename: Yoko
authors.initials: Y
authors.lastname: Tateishi
authors.forename: Mitsuko
authors.initials: M
authors.lastname: Furuya
authors.forename: Fukuo
authors.initials: F
authors.lastname: Kondo
authors.forename: Ikuo
authors.initials: I
authors.lastname: Torii
authors.forename: Kazunori
authors.initials: K
authors.lastname: Nojiri
authors.forename: Yukichi
authors.initials: Y
authors.lastname: Tanaka
authors.forename: Shigeaki
authors.initials: S
authors.lastname: Umeda
authors.forename: Koji
authors.initials: K
authors.lastname: Okudela
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Inayama
authors.forename: Itaru
authors.initials: I
authors.lastname: Endo
authors.forename: Kenichi
authors.initials: K
authors.lastname: Ohashi
mesh_terms: D018248:Adenoma, Liver Cell / Q000098:blood supply / Q000235:genetics* / Q000473:pathology*; D000328:Adult; D005260:Female; D051538:Hepatocyte Nuclear Factor 1-alpha / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D008113:Liver Neoplasms / Q000098:blood supply / Q000235:genetics* / Q000473:pathology*; D011169:Portal Vein / Q000002:abnormalities*
chemical_list: D051538:Hepatocyte Nuclear Factor 1-alpha
keywords: congenital absence of the portal vein; focal nodular hyperplasia; hepatocellular adenomas; hepatocyte nuclear factor (HNF)-1α-inactivated HCA; immunohistochemistry