[Diffuse bile duct papillomatosis: high rate of recurrence and risk of malignant transformation]

Abstract

Bile duct papillomatosis is a rare disease. It is characterised by multicentric papillary lesions of the intra- and/or extrahepatic biliary epithelium. Because of its slow progression, its high rate of recurrence and significant risk of malignant transformation, it is an important cause of obstructive neoplastic bile duct diseases. We report one of these rare cases with the typical clinical signs and exceptional long survival time of eight years: After a history of fifteen years of recurrent epigastric pains the diagnosis of a diffuse biliary papillomatosis was made after surgery. Five years postoperatively a bile duct carcinoma has developed. After a R1-resection of the tumor, the patient was again free of complaints up to the appearances of multiple metastases two years later. He died a year later of metastasizing disease. We discuss the pathological and clinical aspects of this disease and review the literature.