von Meyenburg Complex (Biliary Microhamartoma)



von Meyenburg Complex (Biliary Microhamartoma)


Sanjay Kakar, MD









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

Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on von Meyenburg Complex (Biliary Microhamartoma)

Full access? Get Clinical Tree

Get Clinical Tree app for offline access