Data_Sheet_1_Case Report: Hepatic Adenoma in a Child With a Congenital Extrahepatic Portosystemic Shunt.ZIP

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.

先天性肝外门体分流术（CEPS），亦称 Abernethy 畸形，是一种罕见的畸形，其中肝外门静脉系统直接与下腔静脉相通，从而绕过肝脏。大多数病例中存在门静脉发育不良。CEPS 与肝脏结节的发育有关，范围从主要以局灶性结节性增生（FNH）为主到肝腺瘤（HA）甚至肝细胞癌（HCC）。CEPS 中的肿瘤发育可能归因于灌注压力、氧气供应或内分泌失衡的变化。对于患有肝脏肿瘤的儿童，排除 CEPS 非常重要，因为切除可能会妨碍未来的分流闭塞手术，并且良性肿块在分流闭塞后可能消退。在此，我们回顾了一例 9 岁男性 CEPS 合并肝核因子 1-α（HNF-1-α）失活的 HA 案例以增强对此病症的认识，并回顾了 CEPS 肝脏的病理组织学变化。