Hepatocyte nuclear factor‐1 α inactivated hepatocellular adenomas in patient with congenital absence of the portal vein: A case report

• Published in: Pathology international 2013-07, Vol.63 (7), p.358-363
• Main Authors: Tateishi, Yoko, Furuya, Mitsuko, Kondo, Fukuo, Torii, Ikuo, Nojiri, Kazunori, Tanaka, Yukichi, Umeda, Shigeaki, Okudela, Koji, Inayama, Yoshiaki, Endo, Itaru, Ohashi, Kenichi
• Format: Article
• Language: English
• Subjects: Adenoma, Liver Cell - blood supply; Adenoma, Liver Cell - genetics; Adenoma, Liver Cell - pathology; Adult; congenital absence of the portal vein; Female; focal nodular hyperplasia; hepatocellular adenomas; hepatocyte nuclear factor (HNF)‐1α‐inactivated HCA; Hepatocyte Nuclear Factor 1-alpha - genetics; Humans; Immunohistochemistry; Liver Neoplasms - blood supply; Liver Neoplasms - genetics; Liver Neoplasms - pathology; Portal Vein - abnormalities
• ISSN: 1320-5463; 1440-1827
• DOI: 10.1111/pin.12072

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.