Pancreatobiliary Maljunction: Clinical Features, Diagnosis, and Associated Conditions

Pancreatobiliary maljunction (PBM) is a congenital malformation characterized by the pancreatic and common bile ducts joining anatomically outside the duodenal wall, resulting in the formation of a long common channel. PBM-associated conditions include choledochal cysts and reflux-associated cholecystopathy. Precursor lesions, such as biliary intraepithelial neoplasias, intraductal papillary neoplasms of the bile duct, intracholecystic papillary neoplasms, and cancers associated with PBM, occur in the gallbladder, bile duct, and pancreas. Most PBM cases, along with their associated conditions, precursor lesions, and cancers, have been reported in Asian countries, including Japan and South Korea. However, recent studies have shown no significant difference in frequency between Eastern and Western populations.

To summarize the current understanding of PBMs, as well as recent developments related to associated precursor lesions and cancers.

To understand the clinicopathologic characteristics of PBMs and their associated precursor lesions and cancers, reports from PubMed (US National Library of Medicine) were reviewed.

PBM is a congenital malformation diagnosed primarily by gastroenterologists and radiologists. Pathologists diagnose PBM-associated conditions, including choledochal cysts and reflux-associated cholecystopathy. PBM-related precursor lesions and cancers can develop in the gallbladder, bile duct, and pancreas. Therefore, understanding PBM-associated conditions is crucial for the early detection and effective treatment of patients with PBM-related cancers.