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

Abstract

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.

Update on the Pathology of Pediatric Liver Tumors: A Pictorial Review

“…Patients with congenital extrahepatic portosystemic shunts are also prone to developing HCA, and it is proposed that excess oxygen delivery due to arterialization and increased estrogen and insulin delivery due to diversion of the splanchnic blood flow are the responsible factors [127]. Pediatric Has may also arise in the background of familial adenomatous polyposis syndrome, Fanconi anemia (FA), galactosemia, Hurler syndrome, Alagille syndrome, Abernethy malformation, biliary atresia, congenital hepatic fibrosis, immunodeficiency, cardiac hepatopathy status post-Fontan procedure, germline HNF1A mutations, and maturity-onset diabetes of the young type 3, among others [113,114,128]. HCAs may also occur spontaneously in pediatric settings.…”

Pediatric Primary Hepatic Tumors: Diagnostic Considerations

“…It is theorized that development of HCA is related to the anomalous blood supply characterized by excessive arterialization with resultant increased oxygen delivery to hepatic tissue. In addition, alterations in blood supply to the liver with diversion of splanchnic blood flow may result in abnormal composition of hepatotrophic substances such as insulin and estrogen, which have been clearly linked to development of HCA [ 107 ]. Support for these theories is demonstrated by full or partial regression of HCA after shunt closure and normalization of blood flow [ 108 ].…”