Biliary hamartomas (Von Meyenburg complexes) case report and review of the literature V2 ·...

OBJECTIVE

Biliary hamartomas (von Meyenburg complexes) of theliver are benign lesions. They were first reported byMoschowitz in 1906, but it was Von Meyenburg whodefined them as hamartomatous lesions of bile ducts in19181. They are rare with a prevalence estimated to be0.7%2. Generally, biliary hamartomas are not causingsymptoms and are discovered fortuitously during therealization of imaging evaluations for other pathologiesor during abdominal surgery. However, these lesionsmay mimic metastatic hepatic disease. MRI may help todifferentiate them from metastasis3, however, correctdiagnosis is only based on biopsies andhistopathological analysis.

CONCLUSION

Biliary hamartoma is a rare pathology, most of the timediscovered accidentally. Its presence requires therealization of liver biopsy in order to confirm thediagnosis, but especially in order to exclude acancerous pathology and in particular the presence ofmultiple liver metastasis. Despite its benign character,one must bear in mind the potential risk of developmentof a hepatic cholangiocarcinoma4. Currently, there is norecommendation for follow-up of this pathology.

Figure 1: Initial CT scan with multiple micronodular millimetric calcifications in the liver, without dilations of the intra- and extrahepatic bile ducts.

Biliary hamartomas (Von Meyenburg complexes): case report and review of the literature

Benoit Geng1, Freddy Barros1, Mathias Gugger2, Markus Menth1 and Bernhard Egger1

1Department of Surgery HFR Fribourg - Cantonal Hospital, CH-1708 Fribourg2PROMED Pathology Laboratory, CH-1723 Marly

Figure 2: Peroperative presentation of the liver.

LITERATURE1) Von Meyenburg H. Beitr Pathol Anat 1918;64:4472) Vilgrain V et al. Gastroenterol Clin Biol 2001;25:B1673) Madhusudhan K.S. et al. Indian J Surg. 2009;71:984) Xu AM et al. Eur J Gastroenterol Hepatol. 2009;21:580

METHODS

We report the case of a 73-year-old patient (withcardiopathy, hypertension, diabetes and polyvascularinvolvement), hospitalized in our department for themanagement of an obliterating arteriopathy of thelower limbs stage IV, with realization of an aorto-bi-femoral bypass surgery by median laparotomy. Duringthe preoperative assessment, an angioscanner isperformed on the patient and it fortuitously revealsmultiple micronodular millimetric calcifications in theliver, without dilations of the intra- and extrahepatic bileducts (Fig 1).

RESULTS

The histological analysis of the piece shows thepresence of Von Meyenburg complexes. Thepostoperative evolution afterwards is progressivelyfavorable, allowing a return home at D11.

The dosage of tumor markers shows a very slightincrease in CEA and CA 19.9 with normal alpha-fetoproteins. In peroperative, the liver presents amyriad of yellowish nodules, some of them arecalcified, giving a very heterogeneous and infiltratedaspect of the liver (Fig 2). A biopsy of these lesions forpathological analysis is performed (Fig 3).

Figure 2: Histological analysisA) Biliary hamartoma in a subcapsular localization consisting of irregularly shaped ectatic duct-like structures in a collagenous stroma with multiple calcificatons (insets) and some ilets of preserved liver tissue. Haematoxylin and Eosin. Bar : 0,1 cm. B-C) Insets : Stromal Calcification (arrows). Bile-stained secretion in ectatic duct-like structures (arrowheads).