A bile duct
hamartoma
A hamartoma is a mostly benign, local malformation of cells that resembles a neoplasm of local tissue but is usually due to an overgrowth of multiple aberrant cells, with a basis in a systemic genetic condition, rather than a growth descended fr ...
or biliary hamartoma, is a
benign
Malignancy () is the tendency of a medical condition to become progressively worse.
Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malign ...
tumour
A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...
-like malformation
of the liver.
They are classically associated with
polycystic liver disease
Polycystic liver disease (PLD) usually describes the presence of multiple cysts scattered throughout normal liver tissue. PLD is commonly seen in association with autosomal-dominant polycystic kidney disease, with a prevalence of 1 in 400 to 1000, ...
, as may be seen in the context of
polycystic kidney disease
Polycystic kidney disease (PKD or PCKD, also known as polycystic kidney syndrome) is a genetic disorder in which the renal tubules become structurally abnormal, resulting in the development and growth of multiple cysts within the kidney. These cy ...
, and represent a malformation of the liver plate.
Cause
It is a developmental anomaly in which abnormal tissues are present at normal site.
Due to failure of regression of embryonic biliary duct.
Diagnosis
File:Histopathology of a bile duct hamartoma, high magnification.jpg, Histopathology of a bile duct hamartoma, high magnification, H&E stain. It shows typical features of bile duct hamartoma:
Topic Completed: 23 November 2020. Minor changes: 23 November 2020
- Small to medium sized, irregularly shaped bile ducts lined by bland cuboidal epithelium (may also be flattened).
- Prominent intervening collagenous stroma.
- Bile ducts containing eosinophilic debris (may also contain inspissated bile)
File:Von Meyenburg complex cropped.tif, von Meyenburg Complex in ultrasound. Numerous little cysts with ringdown artefacts.
At
CT scans, bile duct hamartomas appear as small, well-defined hypo- or isoattenuating masses with little or no enhancement after contrast administration.
At
MRI
Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to form pictures of the anatomy and the physiological processes of the body. MRI scanners use strong magnetic fields, magnetic field gradients, and radio waves ...
, they appear hypointense on T1-weighted images, iso- or slightly hyperintense on T2-weighted images, and hypointense after administration of gadolinium based contrast-agent.
On imaging, multiple hamartomas may look similar to metastases or microabscesses.
Treatment
Observation as there is increased risk of cholangiocarcinoma.
Eponym
The eponymous terms (
von Meyenburg complex
A bile duct hamartoma or biliary hamartoma, is a benign tumour-like malformation
of the liver.
They are classically associated with polycystic liver disease, as may be seen in the context of polycystic kidney disease, and represent a malformation ...
, ''Meyenburg complex'') are named for
Hanns von Meyenburg
Hanns von Meyenburg (actually ''Walter'' but called ''Hanns''; 6 June 1887, in Dresden – 6 November 1971) was a Swiss pathologist.
Biography
Hanns von Meyenburg was the son of Swiss sculptor Victor von Meyenburg (1834–1893)01384 Viktor von ...
.
[H. von Meyenburg. Über die Zyztenleber. Beiträge zur pathologischen Anatomie und zur allgemeinen Pathologie, Jena, 1918, 64: 477-532.]
Additional images
Image:Bile duct hamartoma intermed mag.jpg, Micrograph
A micrograph or photomicrograph is a photograph or digital image taken through a microscope or similar device to show a magnified image of an object. This is opposed to a macrograph or photomacrograph, an image which is also taken on a mic ...
of a bile duct hamartoma. Trichrome stain
Trichrome staining is a histological staining method that uses two or more acid dyes in conjunction with a polyacid. Staining differentiates tissues by tinting them in contrasting colours. It increases the contrast of microscopic features in c ...
. Intermediate magnification
Image:Bile duct hamartoma high mag.jpg, Micrograph
A micrograph or photomicrograph is a photograph or digital image taken through a microscope or similar device to show a magnified image of an object. This is opposed to a macrograph or photomacrograph, an image which is also taken on a mic ...
of a bile duct hamartoma. Trichrome stain
Trichrome staining is a histological staining method that uses two or more acid dyes in conjunction with a polyacid. Staining differentiates tissues by tinting them in contrasting colours. It increases the contrast of microscopic features in c ...
, high magnification
Image:Von Meyenburg complex low mag.jpg, Low magnification micrograph
A micrograph or photomicrograph is a photograph or digital image taken through a microscope or similar device to show a magnified image of an object. This is opposed to a macrograph or photomacrograph, an image which is also taken on a mic ...
of a bile duct hamartoma. Trichrome stain
Trichrome staining is a histological staining method that uses two or more acid dyes in conjunction with a polyacid. Staining differentiates tissues by tinting them in contrasting colours. It increases the contrast of microscopic features in c ...
.
Image:Von Meyenburg komplex.jpg, Gross pathologic appearance of a large bile duct hamartoma.
See also
*
Cholestasis
Cholestasis is a condition where bile cannot flow from the liver to the duodenum. The two basic distinctions are an obstructive type of cholestasis where there is a mechanical blockage in the duct system that can occur from a gallstone or malignan ...
*
Ground glass hepatocyte
In liver
The liver is a major organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the synthesis of proteins and biochemicals necessary for digestion and growth. In h ...
*
Mallory body
In histopathology, a Mallory body, Mallory-Denk body, and Mallory's hyaline, is an inclusion found in the cytoplasm of liver cells. Mallory bodies are damaged intermediate filaments within the liver cells.
Associated conditions
Mallory bodies ...
References
Congenital disorders of digestive system
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