Bile duct hamartoma
| Bile duct hamartoma | |
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| Other names: Biliary hamartoma | |
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A bile duct hamartoma or Von Meyenburg complexes[1], 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 of the liver plate.[2]
Signs and symptoms
Bile duct hamartomas are asymptomatic[3]
Cause
Bile duct hamartomas are a developmental anomaly in which abnormal tissues are present at normal site due to failure of regression of embryonic biliary duct.[citation needed]
Diagnosis
At CT scans, bile duct hamartomas appear as small, well-defined hypo- or isoattenuating masses with little or no enhancement after contrast administration.[4] At MRI, they appear hypointense on T1-weighted images, iso- or slightly hyperintense on T2-weighted images, and hypointense after administration of gadolinium based contrast-agent.[4] On imaging, multiple hamartomas may look similar to metastases or microabscesses.[citation needed]
Histopathology of a bile duct hamartoma, high magnification, H&E stain. It shows typical features of bile duct hamartoma:
von Meyenburg Complex in ultrasound. Numerous little cysts with ringdown artefacts.
Micrograph of a bile duct hamartoma. Trichrome stain. Intermediate magnification
Micrograph of a bile duct hamartoma. Trichrome stain, high magnification
Low magnification micrograph of a bile duct hamartoma. Trichrome stain.
Gross pathologic appearance of a large bile duct hamartoma
Treatment
Observation as there is increased risk of cholangiocarcinoma.[citation needed]
Eponym
The eponymous terms (von Meyenburg complex, Meyenburg complex) are named for Hanns von Meyenburg.[5][6]
See also
References
- ↑ "Multiple biliary hamartomas (Concept Id: C4316799) - MedGen - NCBI". www.ncbi.nlm.nih.gov. Archived from the original on 1 September 2023. Retrieved 31 August 2023.
- ↑ Desmet, VJ (Jan 1998). "Ludwig symposium on biliary disorders--part I. Pathogenesis of ductal plate abnormalities". Mayo Clin Proc. 73 (1): 80–9. doi:10.4065/73.1.80. PMID 9443684.
- ↑ Lanser, Haley C.; Puckett, Yana (2023). "Biliary Duct Hamartoma". StatPearls. StatPearls Publishing. Archived from the original on 1 September 2023. Retrieved 31 August 2023.
- ↑ 4.0 4.1 Horton, KM; Bluemke, DA; Hruban, RH; Soyer, P; Fishman, EK (Mar–Apr 1999). "CT and MR imaging of benign hepatic and biliary tumors". Radiographics. 19 (2): 431–51. doi:10.1148/radiographics.19.2.g99mr04431. PMID 10194789.
- ↑ synd/1693 at Who Named It?
- ↑ H. von Meyenburg. Über die Zyztenleber. Beiträge zur pathologischen Anatomie und zur allgemeinen Pathologie, Jena, 1918, 64: 477-532.