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Abstract
Intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) and intraductal papillary mucinous neoplasm of the pancreas (IPMN-P) have striking similarities and are recognized as counterparts. However, simultaneous occurrence of IPMN-B and IPMN-P is extremely rare. A 66 year-old female presented with recurrent epigastric pain and fever. During the past 9 years, she had three clinical episodes related to intrahepatic duct stones and IPMN-P in the pancreas head and was managed by medical treatment. Laboratory test results at admission revealed leukocytosis (12,600/mm3) and elevated CA 19-9 level (1,200 U/mL). Imaging study demonstrated liver abscess in the Couinaud's segment 4, IPMN-B in the left lobe, and IPMN-P in the whole pancreas with suspicious malignant change. Liver abscess was drained preoperatively, followed by left lobectomy with bile duct resection and total pancreatectomy with splenectomy. On histologic examination, noninvasive intraductal papillary mucinous carcinoma arising from various degree of dysplastic mucosa of the liver and pancreas could be observed. However, there was no continuity between the hepatic and pancreatic lesions. This finding in our case supports the theory that double primary lesions are more likely explained by a diffuse IPMN leading to synchronous tumors arising from both biliary and pancreatic ducts rather than by a metastatic process. Herein we present a case of simultaneous IPMN of the bile duct and pancreas which was successfully treated by surgical management.
References
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Fig. 1.
Abdominal CT scans taken before the operation. (A) Abdominal CT scan taken 9 years ago reveals liver abscess at left lateral section with intrahepatic bile duct dilatation (arrowhead) and intraductal papillary mucinous neoplasm of the pancreatic head without malignant features (arrow). (B) Abdominal CT scan taken 4 yeas ago demonstrates marked dilatation of left intrahepatic duct indicating intraductal papillary mucinous neoplasm of the bile duct (arrowhead) and aggravation of intraductal papillary mucinous neoplasm of the pancreas (arrow). (C) Abdominal CT scan taken just before the operation shows aggravated biliary intraductal papillary mucinous neoplasm associated with liver abscess at Couinaud's segment 4 (arrowhead) and mural nodule in pancreas head, indicating malignant change of intraductal papillary mucinous neoplasm of the pancreas (arrow).
Fig. 2.
Evaluation of ampulla of Vater and bile duct. (A) Duodenoscopic view of the ampulla of Vater reveals two “fish-mouth”-shaped patulous openings occupied with thick mucin, each indicating mucin filled bile duct and pancreatic duct. (B) Severe dilatation of the common and left intrahepatic bile duct with amorphous filling defects (arrowheads) is noted on cholangiography performed through percutaneous transhepatic bile drainage catheter. (C) Endoscopic cholangiography carried out after removal of mucin by using basket and balloon shows healthy right intrahepatic duct (arrow).
Fig. 3.
Histologic findings of tumors of the intrahepatic bile duct (A, B) and the pancreatic duct (C, D). Both tumors show variable cytological atypia with no stromal invasion. (A) Intraductal tumor of the liver shows complex branching papillary structure (H&E, ×20). (B) Tumor cells show eosinophilic cytoplasm with moderate amount of apical intracellular mucin and basally located round to oval nuclei (H&E, ×200). (C) Intraductal tumor of the pancreas shows complex branching papillae (H&E, ×20). (D) The tumor cells have elongated and pseudostratified nuclei and show clear cytoplasm with abundant intracytoplasmic mucin (H&E, ×200).
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