|Year : 2016 | Volume
| Issue : 2 | Page : 261-263
Von meyenburg complex: Report of a case and review of literature
N Sadiya1, Sanjeeb K Agrawal2, Mitra Ghosh1, L Thayumanavan3
1 Department of Histopathology, Apollo Speciality Hospital, Chennai, Tamil Nadu, India
2 Department of Radio Diagnosis and Imaging, Apollo Speciality Hospital, Chennai, Tamil Nadu, India
3 Department of Medical Gastroenterology, Apollo Speciality Hospital, Chennai, Tamil Nadu, India
|Date of Web Publication||20-Dec-2016|
Department of Histopathology, Apollo Speciality Hospital, Vanagaram, Chennai, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Von Meyenburg complex (VMC) (biliary hamartoma) is found incidentally in 0.6%–2.8% of adult autopsies or during histological examination and is rarely found in children. They are small, multiple and occur anywhere in the liver. VMC typically causes no symptoms or disturbances in liver functions and in most instances are diagnosed incidentally. They may represent a diagnostic dilemma when liver metastasis is suspected. Given the diagnostic uncertainty over imaging in VMC, liver biopsy is often recommended for a definitive diagnosis. The malignant potential of this finding which is currently considered and frequently ignored raises the necessity of follow-up.
Keywords: Bile duct, liver biopsy, von Meyenburg complex
|How to cite this article:|
Sadiya N, Agrawal SK, Ghosh M, Thayumanavan L. Von meyenburg complex: Report of a case and review of literature. Arch Med Health Sci 2016;4:261-3
|How to cite this URL:|
Sadiya N, Agrawal SK, Ghosh M, Thayumanavan L. Von meyenburg complex: Report of a case and review of literature. Arch Med Health Sci [serial online] 2016 [cited 2017 Aug 22];4:261-3. Available from: http://www.amhsjournal.org/text.asp?2016/4/2/261/196214
| Introduction|| |
The Von Meyenburg complex (VMC) was first described in 1918 and is characterized by multiple bile duct hamartomas. They are described as benign developmental ductal plate malformation of the intrahepatic bile duct., VMC is rare and constitutes typically an asymptomatic finding. Imaging findings, generally incidental, lead to other diagnostic investigations and invasive approaches particularly in oncologic patients. However, there has been a debate in literature with regard to the possible malignant potential of VMC. We present a case of an elderly male in whom an incidental diagnosis of VMC was made on routine health check.
| Case Report|| |
A 56-year-old male patient, a known smoker, with abuse of alcohol for 10 years and with a history of coronary artery disease had come for a routine health check. In view of his personal history of alcohol abuse, he was investigated. Laboratory tests showed normal liver chemistry except for mild elevations of serum bilirubin, alkaline phosphatase, and gamma-glutamyl transpeptidase. Serological tests for hepatitis C virus and HIV were negative. Serum tumor markers revealed normal alpha-fetoprotein. Hematological parameters were within normal limits. A noncontrast computerized tomography (CT) of the abdomen revealed coarse diffuse heterogeneously altered echotexture of liver and ectopic left kidney in left pelvis [Figure 1]a and [Figure 1]b. Radiological impression of cirrhosis/diffuse infiltrative lesions was considered. In view of chronic alcoholism and diffuse parenchymal disease on imaging and normal alpha-fetoprotein, a liver biopsy was done.
|Figure 1: (a and b) A noncontrast computerized tomography scan of the abdomen showing diffuse heterogeneously altered echotexture of liver and ectopic left kidney in left pelvis (as depicted by an arrow).|
Click here to view
Microscopy revealed hepatic tissue showing cord-like arrangement of hepatocytes with bile ducts and portal tract. One of the fragments showed dilated and tortuous bile duct [Figure 2]a and [Figure 2]b. The ducts were lined by low cuboidal type of epithelium with moderate cytoplasm. The ducts were seen to be embedded in a densely fibrous focally hyalinized stroma [Figure 3]. The lumina of the ducts showed the presence of inspissated bile [Figure 3] inset]. No atypical changes were noted. The surrounding hepatocytes appeared unremarkable. Special stain for Masson's trichrome confirmed the presence of fibrosis around the bile ducts [Figure 4]. A diagnosis of VMC (biliary hamartoma) was rendered.
|Figure 2: (a and b) Microphotograph showing linear core of liver tissue with hepatocytes and tortuous bile ducts lined by low cuboidal type of epithelium with moderate cytoplasm (H and E, ×10, ×40).|
Click here to view
|Figure 3: Photomicrograph showing dilated and tortuous bile duct embedded in a densely fibrous stroma (H and E, ×40) and lumina of bile duct showing inspissated bile (Figure 3 inset, as depicted by an arrow).|
Click here to view
|Figure 4: Microphotograph showing special stain–Masson's trichrome, confirming the presence of fibrosis around the bile duct.|
Click here to view
| Discussion|| |
VMC is an uncommon abnormality, and the affected patients are usually asymptomatic. Jaundice may arise as a result of mass effect. Desmet  hypothesized that VMCs arise as a result of arrest or perturbation of the remodeling of the ductal plates that occurs in the late phase of embryologic development of the intrahepatic biliary tree. The biliary hamartoma of VMC may be single or multiple ranging in size from 1 to 15 mm, not all lesions are identifiable on imaging. On ultrasound, VMCs are shown as multiple hyper- or hypo-echoic areas with comet tail echoes. This variation in appearance is due to the presence of cystic lesions, fibrous stroma, and crowded interfaces. The small size of the lesions makes definitive characterization difficult, and lesional reflectivity depends on the size and number of the dilated bile ducts and degree of fibrosis. The lesions are seen as hypodense nodules on noncontrast CT which shows no enhancement on postcontrast imaging. Biliary hamartomas are related to autosomal dominant polycystic kidney disease, Caroli's disease, and congenital hepatic fibrosis. VMCs do not usually cause symptoms or abnormalities in liver function tests but rarely can present as episodes of recurring cholangitis. Multiple bile duct hamartomas can present with symptoms due to portal hypertension due to displacement of the portal vein by the presence of VMC.
Liver biopsy is not contraindicated and should be performed if diagnosis is in doubt. Histologically they are cystically dilated bile ducts lined by single layer of regular cuboidal epithelium embedded in a collagenous stroma. The importance of diagnosing biliary hamartoma is to distinguish these lesions from peribiliary gland hamartoma. Grossly peribiliary gland hamartoma are solitary, well circumscribed, and are microscopically comprised compact network of tubules and acini showing branching, tortuosity, and embedded in a variably fibrous stroma. These lesions are intimately associated with normal portal tract. Unlike biliary hamartoma, the lumens are minimally dilated and do not contain bile. Malignant transformation of these lesions to cholangiocarcinoma has been described, and periodic follow-up has been recommended. Molecular evidence for neoplastic potential of VMC has been recently reported.
The incidence of biliary hamartomas reported in literature varies among the published studies. Chung  found an incidence of 0.69% in a macroscopic examination of 875 consecutive autopsies. In a microscopic surgery of the liver, in 707 consecutive autopsies, Thommesen and Christofferson  reported an incidence of 2.8%. The lower incidence of Chung's macroscopic examination is probably a reasonable estimate of the radiologically detectable hamartoma. VMC has been reported as a rare finding both in clinical practice and in radiological literature because of the asymptomatic presentation of this condition besides the small dimensions and nonrecognition of the lesions.
| Conclusion|| |
VMC is an overall rare finding of the liver. VMCs are usually asymptomatic and do not cause abnormalities in liver function tests but can occasionally present with abdominal symptoms or altered liver function tests. The awareness of this clinicopathological entity is necessary to differentiate VMCs from other diffuse lesions of the liver. In the clinical follow-up of renal cyst in cases of adult polycystic disease, the relevance of screening for VMC is emphasized because of the association between both diseases. Statistical studies are necessary for deeper investigation of the association between VMC and cholangiocarcinoma. It is prudent to follow up such cases in spite of the usual benign behavior of VMC considering the possible malignant potential.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Lev-Toaff AS, Bach AM, Wechsler RJ, Hilpert PL, Gatalica Z, Rubin R. The radiologic and pathologic spectrum of biliary hamartomas. AJR Am J Roentgenol 1995;165:309-13.
Desmet VJ. Congenital diseases of intrahepatic bile ducts: Variations on the theme “ductal plate malformation”. Hepatology 1992;16:1069-83.
Thommesen N, Christoffersen P. Biliary hamartomas (Von Meyenburg complexes) in liver needle biopsies. Acta Pathol Microbiol Scand A 1978;86:93-9.
Xu AM, Xian ZH, Zhang SH, Chen XF. Intrahepatic cholangiocarcinoma arising in multiple bile duct hamartomas: Report of two cases and review of the literature. Eur J Gastroenterol Hepatol 2009;21:580-4.
Wohlgemuth WA, Böttger J, Bohndorf K. MRI, CT, US and ERCP in the evaluation of bile duct hamartomas (Von Meyenburg complex): A case report. Eur Radiol 1998;8:1623-6.
Leuven KU, Desmet VJ. Pathogenesis of ductal plate malformation. J Gastroenterol Hepatol 2004;19:356-60.
Davidoff S, Kim S, Friedman B. Von Meyenburg complexes (bile duct hamartomas). Clin Gastroenterol Hepatol 2006;4:A26.
Zen Y, Terahata S, Miyayama S, Mitsui T, Takehara A, Miura S, et al.
Multicystic biliary hamartoma: A hitherto undescribed lesion. Hum Pathol 2006;37:339-44.
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;24:1131-9.
Jain D, Ahrens W, Finkelstein S. Molecular evidence for the neoplastic potential of hepatic Von Meyenburg complexes. Appl Immunohistochem Mol Morphol 2010;18:166-71.
Chung EB. Multiple bile-duct hamartomas. Cancer 1970;26:287-96.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]