Mucin-producing intrahepatic biliary papillomatosis

Abstract

Purpose

Mucin-producing intrahepatic biliary papillomatosis (MPIBP) is an uncommon tumor. The purpose of this study was to evaluate the clinical, radiological, and histopathological characteristics of MPIBP, and its prognosis.

Methods

A retrospective analysis was conducted of 11 patients who underwent surgery for MPIBP. The clinical features and radiological, pathological, and operative findings were reviewed, and the survival rates were determined.

Results

Repeated episodes of fever and epigastric pain with or without jaundice were the common clinical manifestations. Radiologically, all patients showed diffuse bile duct dilatation with cystic change in intrahepatic bile duct. All patients underwent a hepatic resection with or without an extrahepatic bile duct resection. No in-hospital mortality occurred. All patients survived without any signs of recurrence (median 12 ± 7 months); three patients, including two patients who underwent a palliative resection, had an attack of cholangitis, which was effectively treated with antibiotics.

Conclusions

A diagnosis of MPIBP is usually made in patients with biliary dilatation following a radiologic study. Magnetic resonance cholangiopancreatography is more valuable than other modalities in diagnosis. Mucin-producing intrahepatic biliary papillomatosis is a premalignant disease with high malignant potential. The prognosis of MPIBP is excellent if an aggressive resection is performed. A combination of cholangioscopy and frozen sections during the operation is beneficial for a radical successful surgical resection.