THE DIFFUSE BILE-DUCT PAPILLOMATOSIS - HI GH-RATE OF RECURRENCE AND HIGH-RISK OF MALIGNANT TRANSFORMATION

Bile duct papillomatosis is a rare disease. It is characterised by mul ticentric papillary lesions of the intra- and/or extrahepatic biliary epithelium. Because of its slow progression, its high rate of recurren ce and significant risk of malignant transformation, it is an importan t 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 rec urrent epigastric pains the diagnosis of a diffuse biliary papillomato sis was made after surgery. Five years postoperatively a bile duct car cinoma has developed. After a R1-resection of the tumor, the patient w as again free of complaints up to the appearances of multiple metastas es two years later. He died a year later of metastasizing disease. We discuss the pathological and clinical aspects of this disease and revi ew the literature.