Benign to Malignant Hepatic Lesion Transformation in Abernethy Malformation

Chloe K Tom; Ramon Ter-Oganesyan; Chopra Shefali; Navpreet Kaur; Jordan L Pace; Ali Rastegarpour; Yuri Genyk; Jeffrey A Kahn

doi:10.14309/crj.0000000000001307

Benign to Malignant Hepatic Lesion Transformation in Abernethy Malformation

ACG Case Rep J. 2024 Apr 5;11(4):e01307. doi: 10.14309/crj.0000000000001307. eCollection 2024 Apr.

Authors

Chloe K Tom¹, Ramon Ter-Oganesyan², Chopra Shefali³, Navpreet Kaur⁴, Jordan L Pace⁵, Ali Rastegarpour⁶, Yuri Genyk⁷, Jeffrey A Kahn⁸

Affiliations

¹ Division of Gastrointestinal and Liver Diseases, University of Southern California, Los Angeles, CA, USA.
² Division of Vascular and Interventional Radiology, Department of Radiology, University of Southern California, Los Angeles, CA, USA.
³ Department of Pathology, University of Southern California, Los Angeles, CA, USA.
⁴ Department of Surgery, University of Southern California, Los Angeles, CA, USA.
⁵ California University of Science and Medicine Colton, CA, USA.
⁶ Department of Diagnostic Radiology, University of Southern California Los Angeles, CA, USA.
⁷ Division of Hepatobiliary/Pancreatic and Abdominal Organ Transplant Surgery, University of Southern California Los Angeles, CA, USA.
⁸ Division of Gastrointestinal and Liver Diseases and Liver Transplant Program, University of Southern California Los Angeles, CA, USA.

Abstract

Abernethy malformation or congenital extrahepatic portosystemic shunt is an extremely rare condition whereby the portomesenteric blood drains into a systemic vein and bypasses the liver through a complete or partial shunt. Severe complications include hyperammonemia and encephalopathy, benign and malignant liver tumors, and hepatopulmonary syndrome. We describe a case where a female adult diagnosed with congenital extrahepatic portosystemic shunt subsequently developed focal nodular hyperplasia and then hepatocellular carcinoma.

Keywords: Abernethy malformation; congenital extrahepatic porosystemic shunt; focal nodular hyperplasia; hepatocellular adenoma; hepatocellular carcinoma.

Publication types

Case Reports