Hepatocellular nodules resulting from congenital extrahepatic portosystemic shunts can differentiate into potentially malignant hepatocellular adenomas

• Published in: Journal of hepato-biliary-pancreatic sciences Vol. 22; no. 10; pp. 746 - 756
• Main Authors: Sanada, Yukihiro, Mizuta, Koichi, Niki, Toshiro, Tashiro, Masahisa, Hirata, Yuta, Okada, Noriki, Yamada, Naoya, Ihara, Yoshiyuki, Urahashi, Taizen, Soejima, Yurie, Fukusato, Toshio, Kondo, Fukuo
• Format: Journal Article
• Language: English
• Published: Japan Blackwell Publishing Ltd 01.10.2015; Wiley Subscription Services, Inc
• Subjects: Adenocarcinoma - etiology; Adenocarcinoma - pathology; Adenocarcinoma - surgery; Bile Ducts, Extrahepatic - pathology; Carcinoma, Hepatocellular - etiology; Carcinoma, Hepatocellular - pathology; Carcinoma, Hepatocellular - surgery; Cell Transformation, Neoplastic - pathology; Child; Child, Preschool; Digestive System Abnormalities - diagnosis; Digestive System Abnormalities - surgery; Disease-Free Survival; Female; Focal nodular hyperplasia; Focal Nodular Hyperplasia - etiology; Focal Nodular Hyperplasia - pathology; Focal Nodular Hyperplasia - surgery; Follow-Up Studies; Hepatocellular adenoma; Humans; Japan; Liver cirrhosis; Liver Neoplasms - etiology; Liver Neoplasms - pathology; Liver Neoplasms - surgery; Liver Transplantation - methods; Living Donors; Male; Portasystemic Shunt, Surgical - adverse effects; Portosystemic shunts; Retrospective Studies; Risk Assessment; Sampling Studies; Survival Analysis; Treatment Outcome; Tumors; β-catenin-activated hepatocellular adenoma; Japan; Hepatocellular adenoma; Portosystemic shunts; β-catenin-activated hepatocellular adenoma; Focal nodular hyperplasia
• ISSN: 1868-6974; 1868-6982
• DOI: 10.1002/jhbp.277

Background Hepatocellular nodules caused by congenital extrahepatic portosystemic shunts (CEPS) occur as a result of abnormal portal blood flow, and are mostly cases of benign focal nodular hyperplasia (FNH). However, hepatocellular adenomas (HCA) and hepatocellular carcinomas have been documented in the CEPS patients. HCA can now be immunohistochemically diagnosed; therefore, the concept of hepatocellular nodules resulting from CEPS should be revisited. In this study, we performed a retrospective immunohistochemical investigation of hepatocellular nodules from livers isolated from the CEPS patients undergoing living donor liver transplantation (LDLT). Methods Hepatocellular nodules from livers of five patients with CEPS who underwent LDLT between June 2004 and October 2012 at our institution were immunohistochemically investigated. HCA were classified into four subtypes (HNF1α‐inactivated HCA (H‐HCA); inflammatory HCA; β‐catenin‐activated HCA (b‐HCA); unclassified HCA). Results Sixteen hepatocellular nodules were collected from livers of five patients with CEPS who underwent LDLT. Ten hepatocellular nodules were categorized as FNH (62.5%), five were categorized as b‐HCA (31.3%), and one was categorized as H‐HCA (6.2%). Conclusions Some of the hepatocellular nodules resulting from CEPS were indicative of HCAs, especially the b‐HCA subtype which has the potential for malignant transformation. Surgical or interventional treatments might have to be performed when hepatocellular nodules appear in the CEPS patients. Highlight In this retrospective immunohistochemical investigation of hepatocellular nodules in patients with congenital extrahepatic portosystemic shunts, Sanada and colleagues found that while the majority of nodules were benign focal nodular hyperplasia, over 30% of the nodules were indicative of the β‐catenin‐activated hepatocellular adenoma subtype, which has the potential of malignant transformation.