Benign biliary lesions such as bile duct hamartoma
, bile duct adenoma, and reactive bile ductular proliferation can be difficult to distinguish from metastatic adenocarcinoma (from the pancreas, for example), especially on frozen section (Figure 5, A through C).
Autosomal dominant polycystic liver disease is part of the spectrum of fibropolycystic liver diseases that are caused by congenital bile duct malformation, including bile duct hamartoma, Caroli's disease, congenital hepatic fibrosis, and choledochal cyst.
Also known as von Meyenburg complexes, bile duct hamartomas arise from ductal plate malformations that involve the small interlobular bile ducts.
Bile duct hamartomas (Von Meyenburg complexes): Value of MR imaging in diagnosis.
Case report: Imaging of bile duct hamartomas. Clin Radiol.
(*) AC indicates adenocarcinoma; CIS, carcinoma in situ; in situ; and BDH, bile duct hamartoma.
Cholangiocarcinoma arising in bile duct adenoma with focal area of bile duct hamartoma. Virchows Arch.
This leads to different congenital bile duct disorders, such as Caroli disease and syndrome, autosomal recessive polycystic kidney disease, autosomal dominant polycystic kidney disease, congenital hepatic fibrosis, and bile duct hamartomas (BDHs, also known as von Meyenburg complexes). The clinical presentation and outcome of these congenital diseases show great variation; some patients die as neonates or in early infancy (eg, perinatal, neonatal, and infantile forms of autosomal recessive polycystic kidney disease), and others (eg, BDH) present as clinically asymptomatic and usually incidental findings at laparotomy or autopsy. While approximately 7% of patients with Caroli disease ultimately develop a cholangiocarcinoma, BDH has only rarely been found in association with it.
Cysts and bile duct hamartomas are hypointense in T1 and hyperintense in T2; they are differentiated by a thin ring of gadolinium enhancement, hemangiomas are more common in women, they are hypointense in T1 and moderately hyperintense in T2, with a centripetal enhancement in the arterial phase.
Bile duct hamartomas: diagnostic problems and treatment.