Hepatic von Meyenburg complex simulating biliary cystadenocarcinoma

Area of Science:

• Hepatology
• Gastroenterology
• Pathology

Background:

• Von Meyenburg complex (VMC) comprises benign liver malformations characterized by biliary cystic lesions.
• VMC is often an incidental finding, occurring in 0.6-5.6% of autopsy series.
• Congenital hepatic fibrosis is identified as the cause of ductal plate malformations in VMC.

Observation:

• A rare case of VMC presented with epigastric and right upper quadrant pain, and jaundice.
• Imaging studies (ultrasound, CT, MRI) revealed a periportal multicystic mass.
• The mass mimicked malignant liver conditions such as biliary cystadenoma/cystadenocarcinoma.

Findings:

• Histopathologic examination of a liver wedge biopsy confirmed the diagnosis of VMC.
• VMC can be misdiagnosed as malignant liver disease due to nonspecific imaging findings.
• The patient underwent laparotomy for diagnosis and management.

Implications:

• Accurate diagnosis of VMC is crucial to avoid unnecessary aggressive treatment for presumed malignancy.
• Increased awareness of VMC's presentation can improve diagnostic accuracy.
• Further research into VMC's pathogenesis and optimal diagnostic strategies is warranted.