Abstract
Peribiliary cysts are cystic dilatations resulting from obstruction of extramural peribiliary glands. They are usually benign, may arise from the intrahepatic and extrahepatic bile ducts, and do not communicate with the biliary tree. They may, however, be confused with alternative diagnoses, resulting in unnecessary investigations with profound clinical implications. We present a case of a 42-year-old male, in whom this entity was misdiagnosed as primary sclerosing cholangitis with cholangiocarcinoma.
Highlights
Described by Nakanuma in 1984, peribiliary cysts are benign cystic dilatations that arise as a result of obstruction of extramural peribiliary glands [1]
We present the case of a 42-year-old male misdiagnosed with primary sclerosing cholangitis complicated by cholangiocarcinoma, in whom this diagnosis was definitively confirmed by evaluation of explant liver pathology
As was the case in our patient, misdiagnosis of peribiliary cysts as cholangiocarcinoma in patients with cirrhosis may result in inappropriate delays and even denial of liver transplant consideration in patients who may otherwise be suitable candidates for liver transplantation
Summary
Described by Nakanuma in 1984, peribiliary cysts are benign cystic dilatations that arise as a result of obstruction of extramural peribiliary glands [1] These benign lesions may arise from either intrahepatic or extrahepatic bile ducts and typically have no communication with the biliary tree. A 42-year-old male with a long history of alcohol use was referred for evaluation of possible cholangiocarcinoma complicating underlying primary sclerosing cholangitis with cirrhosis. His clinical presentation was with jaundice, weight loss and imaging evaluation revealed a nodular liver with diffusely beaded appearance of the intrahepatic biliary tree and central hilar prominence suggestive of mass lesion. Multiple cystic biliary structures were noted, lined by non-dysplastic cuboidal biliary type epithelium (Figures 3, 4)
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