Case Report: Hepatic Adenoma in a Child With a Congenital Extrahepatic Portosystemic Shunt

Frontiers in Pediatrics

article

Hannah Glonnegger¹  Simone Hettmer¹  Maren Schulze²  Simone Kathemann²  Sebastian Berg³  Hannah Füllgraf⁴  Andrea Tannapfel⁵  Patrick Gerner¹  Jochen Grohmann⁶  Charlotte Niemeyer¹ 

[1] Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Faculty of Medicine, Medical Center-University of Freiburg, University of Freiburg;Department of Transplant and General Surgery, University Hospital Essen;Division of Pediatric Radiology, Department of Radiology, Faculty of Medicine, Medical Center-University of Freiburg, University of Freiburg;Department of Pathology, Faculty of Medicine, Medical Center-University of Freiburg, University of Freiburg;Faculty of Medicine, Medical Center, Institute for Pathology, Ruhr-University Bochum;Department of Congenital Heart Defects and Pediatric Cardiology, Faculty of Medicine, University Heart Center Freiburg-Bad Krozingen, University of Freiburg

关键词: congenital extrahepatic portosystemic shunt;    hepatic adenoma;    child;    CEPS;    Abernethy malformation;   

DOI  :  10.3389/fped.2020.00501

学科分类：社会科学、人文和艺术（综合）

来源: Frontiers

【 摘 要 】

Congenital extrahepatic portosystemic shunts (CEPS), previously also described as Abernethy malformations, are rare malformations in which the extrahepatic portal system directly communicates with the vena cava inferior, thereby bypassing the liver. A hypoplastic portal vein (PV) exists in most cases. CEPS have been associated with the development of liver nodules, ranging from mostly focal nodular hyperplasia (FNH) to hepatic adenoma (HA) and even hepatocellular carcinoma (HCC). Tumor development in CEPS may be due to changes in perfusion pressures, oxygen supply or endocrine imbalances. It is important to rule out CEPS in children with liver tumors, because resection could impede future shunt occlusion procedures, and benign masses may regress after shunt occlusion. Here, we review the case of a 9-years-old male with CEPS and hepatic nuclear Factor 1-alpha (HNF-1-alpha) inactivated HA to raise awareness of this condition and review histopathological changes in the liver of CEPS.

【 授权许可】

CC BY