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Bile Duct Hamartoma

Synonyms: Meyenburg Complex, Moschcowitz Complex, Ductal Plate Anomaly

Benign glandular proliferation with a fibrous stroma

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Definition

Multiple circumscribed nodules composed of collections of bile ducts in a fibrous stroma.

Pathogenesis

Proposed pathogenetic mechanisms:
- ischemia1
- inflammation
- genetic anomalies

Gross Pathology

Multiple, small, whitish nodules scattered throughout the liver

Histopathology

Nodules:
- a focal disorderly collection of bile ducts and ductules surrounded by abundant fibrous stroma2,3
Pattern of growth of the individual glands may be irregular
Outline of the lesion on low-power view is sharp
No cellular atypia

Other Investigations

If large enough, may be detected ultrasonographically 4

Differential Diagnosis

Select up to 2 differential diagnoses to compare with Bile Duct Hamartoma

Biliary Adenofibroma

A lesion having the general features of bile duct hamartoma but measuring 7cm in diameter has been designated as biliary adenofibroma 5

Metastatic Carcinoma

May be mistaken for metastatic carcinoma by the surgeon6

Genetics

Type	Name	Comment
syndrome	Polycystic disease	In one study, 97% of the patients with polycystic disease of the kidney had bile duct hamartomas

Associated with polycystic disease of kidney and/or liver
- seen in 97% of individuals with adult polycystic renal disease7

Prognosis

Isolated instances of malignant transformation to bile duct carcinomas8,9

References

1 Popovsky MA, Costa JC, Doppman JL. Meyenburg complexes of the liver and bile cysts as a consequence of hepatic ischemia. Hum Pathol. 1979;10:425–432.

2 Chung EB. Multiple bile-duct hamartomas. Cancer. 1970;26:287–296.

3 Thommesen N. Biliary hamartomas (von Meyenburg complexes) in liver needle biopsies. Acta Pathol Microbiol Scand [A]. 1978;86:93–99.

4 Salo J, Bru C, Vilella A, Gines P, Gilabert R, Castells A, et al. Bile-duct hamartomas presenting as multiple focal lesions on hepatic ultrasonography. Am J Gastroenterol. 1992;87:221–223.

5 Tsui WM, Loo KT, Chow LT, Tse CC. Biliary adenofibroma. A heretofore unrecognized benign biliary tumor of the liver. Am J Surg Pathol. 1993;17:186–192.

6 Tsui WM. How many types of biliary hamartomas and adenomas are there?. Adv Anat Pathol. 1998;5:16–20.

7 Redston MS, Wanless IR. The hepatic von Meyenburg complex: prevalence and association with hepatic and renal cysts among 2843 autopsies. Mod Pathol. 1996;9:233–237.

8 Honda N, Cobb C, Lechago J. Bile duct carcinoma associated with multiple von Meyenburg complexes in the liver. Hum Pathol. 1986;17:1287–1290.

9 Jain D, Sarode VR, Abdul-Karim FW, Homer R, Robert ME. Evidence for the neoplastic transformation of Von-Meyenburg complexes. Am J Surg Pathol. 2000;24:1131–1139.

Last updated: 4 Dec 2005