Multiple carcinomata associated with anomalous arrangement of the biliary and pancreatic duct system. A report of two cases with a literature survey.

Two rare cases of multiple neoplasms occurring in association with anomalous arrangement of the pancreatobiliary duct system (PBD) are reported. Ultrasonography, computed tomography and endoscopic retrograde cholangiopancreatography revealed cholecystic tumor in the first case, and multiple choledochocholecystic tumors in the second case. In the first case, pancreatic tumor was discovered two years after cholecystectomy. In the second case, a pancreatic tumor was discovered incidentally in the removed pancreatic head after the operation. The histology of all of these biliary and pancreatic tumors revealed papillary adenocarcinoma in both patients, and additionally small pancreatic endocrine tumors were found in the first case. Postoperative prognosis was comfortably favorable in both patients. In a literature survey, two other cases of double carcinomata associated with anomalous PBD were found, in which the tumors showed the same histology of papillary adenocarcinoma and the postoperative prognoses were also good. These mutual clinicopathological features appear to suggest that this abnormal condition acts as a carcinogenetic risk factor in the pancreatic duct and the biliary duct system, and may finally cause multiple carcinomata in the PBD.