Abstract
Biliary papillomatosis is a rare disease of the biliary tract. In this study, we present a case of a male patient with biliary papillomatosis with malignant change who underwent living donor liver transplantation (LDLT). The 49-year-old man was admitted due to intrahepatic ductal mass, which was finally diagnosed as biliary papillomatosis. It was initially treated by photodynamic therapy, but LDLT was planned due to low efficacy of photodynamic therapy. LDLT was performed with a right liver graft from his son. The pathology report revealed intraductal papillary neoplasm with associated invasive carcinoma. He recovered uneventfully. Although the distal bile duct resection margin was free of tumor, the risk of tumor recurrence at the remnant intrahepatic bile duct still remained. Therefore, staged pylorus-preserving pancreatoduodenectomy was planned. However, after 3 months, he was readmitted due to retropancreatic abscess. It took 6 months to control the intractable retropancreatic abscess. Due to late development of retropancreatic abscess and its slow recovery, staged pancreatoduodenectomy was no longer considered. The patient recovered complete at 1 year posttransplant without any evidence of disease recurrence. After 5 years posttransplant, lung metastasis developed and pulmonary metastasectomy was performed three times. No further recurrence developed during the last 3 years to date, and the patient currently is alive, 10 years after the LDLT. Experience on our case and literature review suggest that patients with biliary papillomatosis with or without malignant transformation are selectively indicated for liver transplantation.