Cyst-forming intraductal papillary neoplasm of the bile ducts: description of imaging and pathologic aspects.

OBJECTIVE: Intraductal papillary neoplasm (IPN) of the bile duct is a newly described pathologic entity characterized by the presence of intraluminal tumors, which sometimes produce a large amount of mucin and form a cystic tumor. Cystic IPN of the bile duct is different from biliary cystadenoma or cystadenocarcinoma in that the former produces intraductal microscopic and macroscopic papillary tumors without ovarian-like stroma, whereas the latter produce a mucin-containing septate cystic tumor without communication with bile duct and with ovarian-like stroma in the cyst wall. The purpose of this study was to evaluate the potential relationships between cyst-forming IPNs of the bile duct and peribiliary glands and also intraductal papillary mucinous neoplasms of the pancreas.

MATERIALS AND METHODS: From a cohort of 87 patients with surgically resected and pathologically proved IPN of the bile duct, 12 patients with cystic IPN of the bile duct who underwent CT (n = 12), MRCP (n = 3), ultrasound (n = 3), and ERCP (n = 4) were included. Imaging findings were evaluated for the relationship of cystic tumors to the bile ducts; in particular, a diverticulum-like appearance was considered as suggestive of the peribiliary gland origin. Pathologic examination was conducted, and both gross and microscopic findings were recorded.

RESULTS: Radiologic examination revealed aneurysm-like dilatation of the involved bile ducts in five patients and intrahepatic biliary cystic tumor in two patients. Interestingly, the remaining five patients had diverticulum-like cystic tumor with or without communication; one patient had a cystic tumor laterally attached to the extrahepatic bile duct. Histopathologically, cystic tumors are lined by atypical biliary epithelium showing intracystic papillary proliferation, with an appearance similar to that of pancreatic intraductal papillary mucinous neoplasm.

CONCLUSION: This study suggests that cyst-forming IPN of the bile duct may be a biliary counterpart to pancreatic intraductal papillary mucinous neoplasm. In particular, at least some of the tumors seem to arise from peribiliary glands, and these cases might be a counterpart to branch-duct intraductal papillary mucinous neoplasm of the pancreas, given the histologic similarity between peribiliary glands and pancreatic branch ducts.