• Annals of surgery · Jul 2022

    Pancreatobiliary Maljunction-Associated Gallbladder Cancer is as Common in the West, Shows Distinct Clinicopathologic Characteristics and Offers an Invaluable Model for Anatomy-Induced Reflux-Associated Physio-Chemical Carcinogenesis.

    • Takashi Muraki, Burcin Pehlivanoglu, Bahar Memis, Michelle D Reid, Takeshi Uehara, Olca Basturk, Jennifer Golia Pernicka, David S Klimstra, William R Jarnagin, Tetsuya Ito, Osamu Hasebe, Shinji Okaniwa, Naoto Horigome, Takeshi Hisa, Pardeep Mittal, Juan M Sarmiento, Shishir K Maithel, Jill Koshiol, Susan Tsai, Douglas Evans, Mert Erkan, and Volkan Adsay.
    • Department of Gastroenterology, Shinshu University Hospital, Nagano, Japan.
    • Ann. Surg. 2022 Jul 1; 276 (1): e32e39e32-e39.

    ObjectiveTo determine the associations of pancreatobiliary maljunction (PBM) in the West.BackgroundPBM (anomalous union of common bile duct and pancreatic duct) is mostly regarded as an Asian-only disorder, with 200X risk of gallbladder cancer (GBc), attributed to reflux of pancreatic enzymes. Methods: Radiologic images of 840 patients in the US who underwent pancreatobiliary resections were reviewed for PBM and contrasted with 171 GBC cases from Japan.ResultsEight % of the US GBCs (24/300) had PBM (similar to Japan; 15/ 171, 8.8%), in addition to 1/42 bile duct carcinomas and 5/33 choledochal cysts. None of the 30 PBM cases from the US had been diagnosed as PBM in the original work-up. PBM was not found in other pancreatobiliary disorders. Clinicopathologic features of the 39 PBM-associated GBCs (US:24, Japan:15) were similar; however, comparison with non-PBM GBCs revealed that they occurred predominantly in females (F/M = 3); at younger (<50-year-old) age (21% vs 6.5% in non-PBM GBCs; P = 0.01); were uncommonly associated with gallstones (14% vs 58%; P < 0.001); had higher rate of tumor-infiltrating lymphocytes (69% vs 44%; P = 0.04); arose more often through adenoma-carcinoma sequence (31% vs 12%; P = 0.02); and had a higher proportion of nonconventional carcinomas (21% vs 7%; P = 0.03). Conclusions: PBM accounts for 8% of GBCs also in the West but is typically undiagnosed. PBM-GBCs tend to manifest in younger age and often through adenoma-carcinoma sequence, leading to unusual carcinoma types. If PBM is encountered, cholecystectomy and surveillance of bile ducts is warranted. PBM-associated GBCs offer an invaluable model for variant anatomy-induced chemical (reflux-related) carcinogenesis.Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.

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