Department of Surgery, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-8513, Japan.
Department of Pathology and Laboratory Medicine, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-0042, Japan.
World J Surg Oncol. 2020 Nov 9;18(1):292. doi: 10.1186/s12957-020-02072-7.
Pancreaticobiliary maljunction (PBM) is a congenital abnormality in which the pancreatic and biliary ducts join anatomically outside the duodenal wall resulting in the regurgitation of pancreatic juice into the biliary tract (pancreatobiliary reflux). Persistent pancreatobiliary reflux causes injury to the epithelium of the biliary tract and promotes the risk of biliary cancer. Intracholecyctic papillary neoplasm (ICPN) has been highlighted in the context of a cholecystic counterpart of intraductal papillary mucinous neoplasm of the pancreas and the bile duct, but the tumorigenesis of ICPNs remains unclear.
A 52-year-old Japanese woman was referred for the assessment of dilation of the bile duct. Computed tomography which revealed an enhanced mass in the gallbladder and endoscopic retrograde cholangiopancreatography confirmed that the confluence of the main pancreatic duct and extrahepatic bile duct (EHBD) was located outside the duodenal wall. Under the diagnosis of gallbladder cancer with PBM, cholecystectomy with full thickness dissection, EHBD resection, lymph node dissection, and hepaticojejunostomy were performed. Macroscopic examination of the resected specimen showed that the cystic duct was dilated and joined into the EHBD just above its confluence with the pancreatic duct, and the inflamed change of non-tumorous mucosa of gallbladder indicating that there was considerable mucosal injury due to pancreatobiliary reflux to the gallbladder. Histopathological examination revealed that the gallbladder tumor was a gastric-type ICPN with non-invasive component. Either KRAS gene mutation or p53 protein expression that were known to be associated with the carcinogenesis of biliary cancer under the condition of pancreatobiliary reflux was not detected in the tumor cells of ICPN.
The present case might suggest that there was no association between PBM and ICPN. To reveal the tumorigenesis of ICPN and its attribution to pancreatobiliary reflux, however, further study is warranted.
胰胆管合流异常（PBM）是一种先天性异常，其特征是胰管和胆管在十二指肠壁外解剖性汇合，导致胰液反流至胆道（胰胆反流）。持续性胰胆反流会损伤胆道上皮，并增加胆道癌的风险。胆囊内乳头状肿瘤（ICPN）在胰腺和胆管内的导管内乳头状黏液性肿瘤的胆囊对应物的背景下被强调，但 ICPN 的肿瘤发生机制仍不清楚。
一位 52 岁的日本女性因胆管扩张而就诊。计算机断层扫描显示胆囊内增强肿块，内镜逆行胰胆管造影证实主胰管和肝外胆管（EHBD）汇合位于十二指肠壁外。根据 PBM 伴胆囊癌的诊断，行胆囊切除术、全层剥离、EHBD 切除、淋巴结清扫和胆肠吻合术。切除标本的大体检查显示胆囊管扩张，在其与胰管汇合的上方与 EHBD 汇合，胆囊非肿瘤性黏膜的炎症改变表明由于胰胆反流对胆囊有相当大的黏膜损伤。组织病理学检查显示胆囊肿瘤为胃型 ICPN，具有非浸润性成分。在胰胆反流的情况下与胆管癌发生相关的 KRAS 基因突变或 p53 蛋白表达在 ICPN 肿瘤细胞中均未检测到。
本病例提示 PBM 与 ICPN 之间无关联。然而，为了揭示 ICPN 的肿瘤发生机制及其与胰胆反流的关系，需要进一步研究。
