title: Hepatocellular nodules resulting from congenital extrahepatic portosystemic shunts can differentiate into potentially malignant hepatocellular adenomas.
abstract: 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.
authors.affiliation: Department of Transplant Surgery, Jichi Medical University, Shimotsuke City, Japan.
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Sanada
authors.affiliation: Department of Transplant Surgery, Jichi Medical University, Shimotsuke City, Japan.
authors.forename: Koichi
authors.initials: K
authors.lastname: Mizuta
authors.affiliation: Department of Pathology, Jichi Medical University, Shimotsuke City, Japan.
authors.forename: Toshiro
authors.initials: T
authors.lastname: Niki
authors.affiliation: Department of Transplant Surgery, Jichi Medical University, Shimotsuke City, Japan.
authors.forename: Masahisa
authors.initials: M
authors.lastname: Tashiro
authors.affiliation: Department of Transplant Surgery, Jichi Medical University, Shimotsuke City, Japan.
authors.forename: Yuta
authors.initials: Y
authors.lastname: Hirata
authors.affiliation: Department of Transplant Surgery, Jichi Medical University, Shimotsuke City, Japan.
authors.forename: Noriki
authors.initials: N
authors.lastname: Okada
authors.affiliation: Department of Transplant Surgery, Jichi Medical University, Shimotsuke City, Japan.
authors.forename: Naoya
authors.initials: N
authors.lastname: Yamada
authors.affiliation: Department of Transplant Surgery, Jichi Medical University, Shimotsuke City, Japan.
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Ihara
authors.affiliation: Department of Transplant Surgery, Jichi Medical University, Shimotsuke City, Japan.
authors.forename: Taizen
authors.initials: T
authors.lastname: Urahashi
authors.affiliation: Department of Pathology, Teikyo University School of Medicine, Itabashi City, Japan.
authors.forename: Yurie
authors.initials: Y
authors.lastname: Soejima
authors.affiliation: Department of Pathology, Teikyo University School of Medicine, Itabashi City, Japan.
authors.forename: Toshio
authors.initials: T
authors.lastname: Fukusato
authors.affiliation: Department of Pathology, Teikyo University School of Medicine, Itabashi City, Japan.
authors.forename: Fukuo
authors.initials: F
authors.lastname: Kondo
mesh_terms: D000230:Adenocarcinoma / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D017734:Bile Ducts, Extrahepatic / Q000473:pathology*; D006528:Carcinoma, Hepatocellular / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D002471:Cell Transformation, Neoplastic / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D004065:Digestive System Abnormalities / Q000175:diagnosis / Q000601:surgery; D018572:Disease-Free Survival; D005260:Female; D020518:Focal Nodular Hyperplasia / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D007564:Japan; D008113:Liver Neoplasms / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D016031:Liver Transplantation / Q000379:methods; D019520:Living Donors; D008297:Male; D011170:Portasystemic Shunt, Surgical / Q000009:adverse effects; D012189:Retrospective Studies; D018570:Risk Assessment; D012494:Sampling Studies; D016019:Survival Analysis; D016896:Treatment Outcome
keywords: Focal nodular hyperplasia; Hepatocellular adenoma; Portosystemic shunts; β-catenin-activated hepatocellular adenoma