Department of Gastroenterology, Shinshu University Hospital, Nagano, Japan.
Department of Pathology, Emory University Hospital, Atlanta, Georgia.
Ann Surg. 2022 Jul 1;276(1):e32-e39. doi: 10.1097/SLA.0000000000004482. Epub 2020 Nov 12.
To determine the associations of pancreatobiliary maljunction (PBM) in the West.
PBM (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.
Eight % 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.
确定西方胰胆管合流异常（PBM）的关联。
PBM（胆总管和胰管异常连接）主要被认为是亚洲特有的疾病，其胆囊癌（GBc）的风险增加 200 倍，归因于胰酶反流。方法：回顾性分析美国 840 例接受胰胆管切除术患者的放射影像学图像，以确定 PBM，并与日本 171 例胆囊癌病例进行对比。
美国 8%的胆囊癌（24/300）存在 PBM（与日本相似，15/171，8.8%），此外还有 1/42 例胆管癌和 5/33 例胆总管囊肿。美国的 30 例 PBM 病例中，没有一例在最初的检查中被诊断为 PBM。其他胰胆管疾病中未发现 PBM。美国的 39 例 PBM 相关胆囊癌（24 例）和日本的 15 例胆囊癌患者的临床病理特征相似；然而，与非 PBM 胆囊癌相比，它们主要发生在女性（F/M=3）；年龄较轻（<50 岁）（21%与非 PBM 胆囊癌的 6.5%相比；P=0.01）；罕见与胆石症相关（14%与 58%相比；P<0.001）；肿瘤浸润淋巴细胞比例较高（69%与 44%相比；P=0.04）；更多通过腺瘤-癌序列发生（31%与 12%相比；P=0.02）；非典型癌比例较高（21%与 7%相比；P=0.03）。结论：PBM 也占西方胆囊癌的 8%，但通常未被诊断。PBM-GBC 往往在较年轻的年龄发病，并且经常通过腺瘤-癌序列发生，导致不常见的癌类型。如果遇到 PBM，应进行胆囊切除术和胆管监测。PBM 相关的胆囊癌为变体解剖结构引起的化学（反流相关）致癌提供了一个非常有价值的模型。
