von Meyenburg Complex (Biliary Microhamartoma)
Sanjay Kakar, MD
Key Facts
Terminology
Developmental malformation resulting from aberrant construction/remodeling of embryonic bile ducts
Etiology/Pathogenesis
Most often sporadic; also associated with several fibropolycystic liver diseases
Macroscopic Features
Usually multifocal and < 0.5 cm
Microscopic Pathology
Angulated, branching, irregular ducts embedded in dense fibrous stroma
Usually within or at edge of portal tracts
Diagnostic Checklist
Multiple widely scattered VMC raise possibility of associated fibropolycystic disease
 This small, subcapsular VMC features branching, irregular, angulated glands embedded in a dense fibrous stroma. |
 The branching, angulated glands in VMC are lined by a single layer of flattened cuboidal epithelium. There is no nuclear atypia. |
TERMINOLOGY
Abbreviations
von Meyenburg complex (VMC)
Synonyms
Biliary microhamartoma
Ductal plate malformation
Definitions
Developmental malformation resulting from aberrant construction/remodeling of embryonic bile ducts
ETIOLOGY/PATHOGENESIS
Developmental Anomaly
Within spectrum of fibropolycystic diseases of liver
Most often sporadic
Also associated with numerous fibropolycystic diseases including congenital hepatic fibrosis, Caroli disease, autosomal dominant polycystic disease
Considered precursor lesion of autosomal dominant polycystic liver disease
Basic lesion of congenital hepatic fibrosis
