Monolobar Hepatobiliary Fibropolycystic Disease

Abstract

We herein report a case of monolobar hepatobiliary fibropolycystic disease. A 75-year-old woman presented with heartburn. Imaging modalities including US, CT, and MRI revealed marked atrophy and multiple biliary cysts of the hepatic left lobe. The hepatic right lobe was normal. ERCP and bile duct endoscopy revealed anomalous pancreaticobiliary union, choledochal dilation, dilation of left intrahepatic bile ducts, and small choledochal non-invasive adenocarcinoma. Polycystic kidney diseases were absent. The patient underwent pancreatico-duodenectomy and extended hepatic left lobectomy. Grossly, the hepatic left lobe was markedly atrophic, and studded with numerous biliary cysts. The left intrahepatic bile ducts were dilated (Caroli's disease) and the common bile duct showed type I choledochal dilation. The right hepatic lobe was normal. Histologically, the hepatic left lobe was replaced by fibroelastosis. The intrahepatic bile ducts showed ductal plate malformation such as irregular contours, invaginations, and protrusions. The numerous biliary cysts also showed ductal plate malformation. There were numerous persistent ductal plates and microhamartomas. Many hyalinized destructive biliary cysts and ductal plates were recognized. The liver parenchyma was scant and free of hepatocellular malformations. The portal veins showed old obliterative portal thrombosis. The right hepatic lobe was normal. Immunohistochemically, the biliary cells were positive for cytokeratin 7, 8, 18 and 19, and MUC6 and CD10, but negative for MUC2 and MUC5AC. The biliary cysts, persistent ductal plate, and microhamartomas were positive for fetal apomucin antigen MUC1.

Congenital Hepatic Fibrosis and Autosomal Recessive Polycystic Kidney Disease

“…The patient died 2 months later. The case of monolobar (left-sided) Caroli disease described by Terada et al (18) was remarkable for extrahepatic cholangiocarcinoma. The patient described also had recurrent cholangitis.…”

Monosegmental Hepatobiliary Fibropolycystic Disease Mimicking a Mass: Report of Three Cases

“…Ductal plate malformations can be involved monosegmentally or monolobarly during the process of bile duct development, as well Caroli's disease (3, 4). In the present cases, ductal plate malformations were involved monosegmentally, and the remaining liver was normal.…”

“…Ductal transformation of hepatocytes is unlikely in the present case, but may be due to fetal biliary antigen. Examination of CD10 in the fetal ductal plate is mandatory (3, 4). …”

“…These diseases are characterized by varying degrees of persistent embryonic bile duct structures, fibrosis, and ductal dilatation (2). Congenital biliary malformations, including adult and infantile polycystic disease of the liver and kidneys, congenital hepatic fibrosis, Caroli's disease, von Meyenburg complexes, and choledochal cyst have been collectively termed ductal plate malformations or hepatobiliary fibropolycystic diseases (3-6). However, the present cases did not belong to, and were different from, the above described malformations.…”

Human ductal plate and its derivatives express antigens of cholangiocellular, hepatocellular, hepatic stellate/progenitor cell, stem cell, and neuroendocrine lineages, and proliferative antigens

“…Such structures are called DP malformations (DPM) or hepatobiliary fibropolycystic disease, which involves congenital hepatic fibrosis, polycystic diseases (adult and infantile) of the liver and kidneys, congenital biliary atresia, von-Meyenburg complex, and Caroli's disease. [18][19][20][21][38][39][40][41][42][43] DP is also seen in ductular reactions [44][45][46] and DPM in some liver hamartoma 45 and cholangiocarcinoma. 47 The DP in ductular reaction of focal nodular hyperplasia interestingly may express KIT, 46 a receptor of stem cell factor (SCF).…”