Von Meyenburg Complexes and Malignancy: A Systematic Review

Abstract

ABSTRACTVon Meyenburg Complexes (VMC) are benign lesions secondary to ductal plate malformations. These are relatively rare in adults and are often asymptomatic, with many lesions being diagnosed on imaging. Despite these benign characteristics, VMC have the potential to undergo malignant changes leading to conditions such as cholangiocarcinoma and other cancers. The objective of the review is to report and analyze the cases of biliary hamartomas which were associated with malignancy. We performed a structured systematic review of literature and identified 31 cases of biliary duct hamartomas associated with malignancy. The mean age at onset was 61 years±13 (min 19 to max 88) with the majority of the patients being male (64.5%). Of all cases, 41.9% reported symptoms (13/31 cases) with the most common symptom being abdominal pain (32.3%). Biopsies reported malignancy in 77.4% of cases (24/31 cases) with the most common reported associated malignancy being cholangiocarcinoma 54.8% (17/31). 93.3% of the cases underwent surgical intervention (28/30 cases). VMC are an important entity with malignancy potential which is under-reported. Presence of ‘red flag’ symptoms such as weight loss and obstructive jaundice in patients with diagnosed biliary hamartomas should prompt an evaluation for malignancy.