Clinical features of pancreaticobiliary maljunction: update analysis of 2nd Japan‐nationwide survey

Abstract

Introduction Pancreaticobiliary maljunction (PBM) is a congenital anomaly, which can be defined as a union of the pancreatic and biliary ducts located outside off the duodenal wall. We herein investigate clinical features of PBM including as the 2nd report of a Japanese nationwide survey.Patients and methods During a period of 18 years (from 1990 to 2007), 2,561 patients with PBM were registered at 141 medical institutions in Japan. Among them, eligible patients (n = 2,529) were divided into two groups: adult (n = 1,511) and pediatric patients (n = 1,018). Comparisons of clinical features including associated biliary cancers were performed according to the biliary dilatation (BD), age factor, and time era. Results Only one case in pediatric patients with BD combined with a bile duct cancer (0.1 %). In adult patients, the bile duct cancer and the gallbladder cancer was seen in 6.9 and 13.4 % patients with BD and in 3.1 and 37.4 % patients without BD, respectively. In adult patients with BD, the occurrence rates of biliary cancers were increased in latter period (00'-07') compared with former period (90'-99'). The ratio of biliary cancer localization was changed between former and latter period, and the bile duct cancer was increased in latter period (from 5.5 to 9.3 %).Conclusions The largest series of PBM were evaluated to clarify the clinical features including the associated biliary cancer in this Japan-nationwide survey. This report could be widely used in the future as a reference data for diagnosis and treatment of PBM.

Pancreaticobiliary maljunction and biliary cancer

“…The ratio of gallstone detection in PBM patients who developed gallbladder cancer was lower than that in the biliary cancer population without PBM [2,3,32]. In our series, the ratios were 10 % and 62 %, respectively [1].…”

“…According to a nationwide survey in Fig. 1 The pathophysiology of pancreaticobiliary maljunction [3] Japan (n = 2561) [2], biliary cancer was detected in 21.6 % of adult patients with congenital biliary dilatation and in 42.4 % of PBM patients without biliary dilatation. In patients with biliary cancers in association with PBM, the location ratio of cancers in the bile duct and gallbladder were 32.1 % and 62.3 % in congenital biliary dilatation, and 7.3 % and 88.1 % in PBM patients without biliary dilatation, respectively.…”

“…Prophylactic cholecystectomy is performed in many institutes, as most biliary cancers that develop in PBM patients without biliary dilatation are gallbladder cancers [38,39]. However, some surgeons propose excision of the extrahepatic bile duct together with the gallbladder for PBM patients without biliary dilatation [23], because the frequency of bile duct cancer in PBM patients without biliary dilatation is higher compared to that in the general population [2], and K-ras and/or p53 gene mutations are also reportedly seen in the bile duct of PBM patients without biliary dilatation [23,40].…”

“…1). PBM can be divided into PBM with biliary dilatation (congenital biliary dilatation) and PBM without biliary dilatation [1,2].…”

Clinical practice guidelines for the management of biliary tract cancers 2015: the 2nd English edition

“…A Japanese retrospective nationwide survey of PBM revealed that the incidence of biliary tract cancers was 21.6% in the group with bile duct dilatation; 13.4% were gallbladder carcinoma, 7.0% were bile duct carcinoma, and 1.0% were both gallbladder and bile duct carcinomas [20]. According to the result and other reports, the prophylactic excision of the gallbladder and common bile duct is the appropriate surgical procedure for PBM with bile duct dilatation [21].…”