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

Abstract

Hepatobiliary fibropolycystic diseases are a unique group of entities involving the liver and biliary tract, which are caused by abnormal embryologic development of the ductal plates at various stages. We experienced strange hepatobiliary fibropolycystic diseases with a complex mass composed of malformed ducts and biliary cysts, which did not belong to, and were different from, previously known malformations. They were unique in imaging and histologic features. We herein report three cases of monosegmental hepatobiliary fibropolycystic disease mimicking a mass.

Congenital Hepatic Fibrosis and Obliterative Portal Venopathy Without Portal Hypertension - A Review of Literature Based on an Asymptomatic Case

“…Congenital hepatic fibrosis (CHF) is an autosomal recessive disease (1,2) , due to a mutation on PKHD1, a gene encoding fibrocystin/ polyductine (1,2,3) , a ciliary protein expressed in cholangiocytes. CHF is defined by varying degrees of periportal fibrosis and irregular proliferation of bile ducts (1,4,5) , being part of the so-called Fibropolycystic Diseases, which also include Caroli's Disease (6) , Autosomal Dominant Renal Disease, Autosomal Recessive Polycystic Kidney Disease, Von Meyenburg Complex (bile duct hamartoma), and choledochal cyst (5,7,8) . Typically, CHF patients have been diagnosed in infancy or early childhood, but some recent data demonstrate that some patients remain asymptomatic for long periods (4) , as the presented case here.…”

Choledochal cysts: sequel of the malformed embryological ductal plate?

“…A partial or complete arrest of normal remodeling of the embryological ductal plate results in persistence of embryonic cystic structures ( Fig. 2) constituting what is known as fibro-polycystic liver disease or the ductal plate malformations [4,5]. During embryogenesis, it is the level of insult (intrahepatic small, medium, or large-sized bile ducts and/or involvement of the extrahepatic biliary tree) that determines the type of ductal plate disorder (Fig.…”

“…The embryological ductal plate is a double-layered tubular column of precursor hepatoblasts which surround the portal vein radicle and progressively remodel to form the future bile ducts ( Fig. 1) [2][3][4][5][6]. A partial or complete arrest of normal remodeling of the embryological ductal plate results in persistence of embryonic cystic structures ( Fig.…”

“…the embryological ductal plate [2][3][4][5]. The embryological ductal plate is a double-layered tubular column of precursor hepatoblasts which surround the portal vein radicle and progressively remodel to form the future bile ducts ( Fig.…”

“…3) [2][3][4][5][6]. Choledochal cysts are considered to be a ductal plate malformation of the extrahepatic bile duct with or without intrahepatic bile duct involvement [2][3][4][5].…”