Intrahepatic biliary papillomatosis:a report of 9 cases / 中华肝胆外科杂志

ABSTRACT

Objective Intrahepatic biliary papillomatosis(IBP)is a rare disease that is characterized by multiple numerous papillary adenomas in the intrahepatic biliary duct(IBD).The clinical features and outcome,however,are not well known.The clinicopathologic features,treatments and follow-up results were retrospectively analyzed in order to improve the efficiency of diagnosis and treatment for the disease.Methods Between August 2006 and October 2008,9 patients were diagnosed with IBP by histological findings at a tertiary referral center,Ningbo Medical Center(University of Ningbo,College of Medicine,Ningbo,China).The authors retrospectively reviewed the medical records to obtain clinical,radiological and pathologic data.The therapeutic results and follow-up data were also reviewed.Results The ratio of male to female was 27 and the middle age was 59 years.Repeated episodes of fever and right upper quadrant abdominal pain with or without jaundice were the common clinical manifestations.There were intrahepatic choledocholithiasis and/or history of previous biliary operation in 8 cases.The level of CA19-9 and CEA were almost normal.Imaging workup demonstrated cyst-like dilatation of intrahepatic biliary tree with or without liver atrophy,which were more obvious in the mucin-hypersecreting IBP.All of the 9 cases underwent curative resection with an adequate resection margin.Macroscopic findings demonstrated that the tumors of 9 cases were all located in the left lobe with mucin-hypersecreting type in 7 cases.The diameter of numerous papillary granular was usually less than 10 mm(from 2 to 5 mm.frequently)and friable,that filled the dilated IHD dispersive.Fine fibrovascular cores lined by dysplastic epithelial cells were frequently found under microscope.Few foci of stromalinvasion were noticed in the two cases with malignant transformation.All of the 9 cases survived and there was no recurrence.Conclusion IBP occurs more often in middle and old women who have history of biliary disease. Repeated episodes of cholangitis are the common clinical manifestations. Extra- and intrahepatic biliary tract dilatation is the common imaging finding.MRCP/ERCP is more valuable than others in diagnosis. Clinical and histological finding shows that IBP is a premalignant disease with high malignant potential. Curative resection should be done as earlyas possible for the long-term survival rate. The use of cholangioscopy in operation and rapid biopsy of resection margin will benefit the curative resection.