Bile duct hamartoma

by Alexandra States, MD and Stephanie Reid, MD FRCPC
March 7, 2023

What is a bile duct hamartoma?

A bile duct hamartoma, also known as a von Meyenburg complex, is a non-cancerous liver tumour made up of disorganized bile ducts.  It is a common type of liver tumour seen in approximately 3-6% of the population. Some may appear before birth during the time in fetal development when the liver and bile ducts are first forming.

What are the symptoms of a bile duct hamartoma?

Bile duct hamartomas do not cause any symptoms and are usually found during an operation for an unrelated issue or at autopsy. Most are small (less than 0.5 cm) and are located just below the covering of the liver (liver capsule). When examined without a microscope most look like a grey, white, or green spot on the surface of the liver.

What causes a bile duct hamartoma?

Most bile duct hamartomas are called sporadic because they have no known cause.

How do pathologists make the diagnosis of bile duct hamartoma?

Pathologists make this diagnosis by looking at a tissue sample from the liver under a microscope. The tissue sample is usually removed in a procedure called a biopsy or a resection. If the tumour is discovered during an operation, your surgeon may ask a pathologist to look at a tissue sample from the tumour under the microscope. This is called a frozen section or intraoperative consultation.  This procedure is performed because it can be difficult to tell the difference between a bile duct hamartoma and cancer without a microscope.

What does a bile duct hamartoma look like under the microscope?

When viewed under the microscope, a bile duct hamartoma looks like an abnormal collection of bile ducts surrounded by the normal liver.  The bile ducts in a hamartoma are often larger and less round than normal bile ducts. Some of the abnormal ducts may be filled with bile. However, unlike normal bile ducts, the ducts in a hamartoma are not connected to the biliary tree so bile cannot flow from these ducts out of the liver.

What conditions are associated with multiple bile duct hamartomas?

Fibrocystic liver disease is a condition where a person develops abnormal spaces in the liver called cysts. People with fibrocystic liver disease may also develop multiple bile duct hamartomas. Other conditions associated with multiple bile duct hamartomas include congenital hepatic fibrosis and Caroli disease.

What is the treatment for a bile duct hamartoma?

No treatment is necessary for most bile duct hamartomas. However, if your doctor believes you may have a condition, such as fibrocystic liver disease, he or she may offer you treatment.