• Developmental malformation resulting from aberrant construction/remodeling of embryonic bile ducts
Majority are sporadic
Also associated with several fibropolycystic liver diseases
Clinical Issues
• Reportedly occurs in ∼ 3% of autopsied patients
• Not considered premalignant
Macroscopic
• Usually multifocal
• Often subcapsular
• Typically < 0.5 cm
Microscopic
• Angulated, branching, irregular ducts embedded in dense fibrous stroma
Usually within or at edge of portal tracts
Ducts are lined by cuboidal, often flattened, epithelium
Ducts may contain eosinophilic proteinaceous debris or inspissated bile
Diagnostic Checklist
• Multiple widely scattered von Meyenburg complexes raise possibility of associated fibropolycystic disease
• May be submitted for frozen section during abdominal surgery to exclude liver metastasis
von Meyenburg Complex, Low Power This small, subcapsular von Meyenburg complex (VMC) features branching, irregular, angulated glands embedded in dense fibrous stroma.
von Meyenburg Complex, Medium Power VMC is characterized by angulated, branching glands within dense, fibrous stroma. Note the inspissated bile . There is no nuclear atypia and no mitoses.
von Meyenburg Complex, High Power The branching, angulated glands in VMC are lined by a single layer of flattened cuboidal epithelium. There is no nuclear atypia.
von Meyenburg Complex and Polycystic Liver Disease Some of the glands within VMC have dilated to produce a cyst , which is filled with eosinophilic proteinaceous material. This patient has autosomal dominant polycystic disease.
TERMINOLOGY
Abbreviations
• von Meyenburg complex (VMC)
Synonyms
• Biliary microhamartoma
• Ductal plate malformation
Only gold members can continue reading. Log In or Register to continue
. There is no nuclear atypia and no mitoses.
, which is filled with eosinophilic proteinaceous material. This patient has autosomal dominant polycystic disease.
