Published online Jan 6, 2019. doi: 10.12998/wjcc.v7.i1.102
Peer-review started: September 27, 2018
First decision: November 2, 2018
Revised: December 6, 2018
Accepted: December 7, 2018
Article in press: December 8, 2018
Published online: January 6, 2019
Processing time: 99 Days and 3.6 Hours
Intraductal papillary neoplasm of the bile duct (IPNB) is pathologically similar to intraductal papillary mucinous neoplasm (IPMN). However, there are several significant differences between them. The rate of IPMN associated with extrapancreatic malignancies has been reported to range from 10%-40%, and it may occasionally be complicated with the presence of fistulas. IPMN associated with malignant IPNB is extremely rare and only nine cases have been reported in the literature.
We report a 52-year-old man who presented with recurrent cholangitis for nine months. Computed tomography and magnetic resonance cholangiopancreatography showed the common bile duct stricture with dilated pancreatobiliary duct without other abnormal findings. The underlying pathogenesis could not be identified based on the radiologic images. Endoscopic retrograde cholangiopancreatography revealed a pancreatobiliary fistula with dilated main pancreatic duct, biliary stricture with dilated biliary tree, and mucus discharge from the enlarged orifice of the major papilla. The patient underwent SpyGlass cholangiopancreatoscopy due to a suspected mucin-producing biliary neoplasm and indeterminate main pancreatic duct dilatation. Multiple papillary growing neoplasms with vascular images, with the extent of lesions spreading in the biliopancreatic ductal lumens, were identified by SpyGlass. In addition, the presence of a pancreatobiliary fistula was also identified. The patient was diagnosed as having benign IPMN and malignant IPNB with focal invasion by postoperative pathology. Furthermore, varying histological subtypes were present in both IPMN and IPNB. Pylorus-preserving pancreaticoduodenectomy was performed on the patient with excellent results during the 52 month follow-up period.
We deemed that pancreatography and SpyGlass allowed for an efficient diagnosis of IPMN with pancreatobiliary fistula, whereas the etiology could not be identified by radiologic imaging.
Core tip: We report a patient with an extremely rare co-occurrence of intraductal papillary mucinous neoplasm (IPMN) and malignant intraductal papillary neoplasm of the bile duct (IPNB) accompanied with a pancreatobiliary fistula. The etiology of biliopancreatic duct dilatations could not be identified by radiologic imaging. After the patient underwent endoscopic retrograde cholangiopancreatography, the presence of a pancreatobiliary fistula was revealed by pancreatography. SpyGlass cholangiopancreatoscopy showed multiple papillary neoplasms in the pancreaticobiliary duct. In addition, SpyGlass was able to identify a pancreatobiliary fistula. The patient was diagnosed with benign IPMN and malignant IPNB. Different histological subtypes for both IPMN and IPNB were also identified. Radical resection on the patient achieved excellent results.