Malignancy of intraductal papillary neoplasm of the bile duct.

The intraductal papillary neoplasm of the bile duct (IPNB) is an uncommon disease which was first included in the World Health Organization classification of neoplasms in 2010. Although some patients may be asymptomatic, symptoms include most frequently abdominal pain, cholangitis and jaundice. Contrary to the pancreatic intraductal papillary mucinous neoplasm, this entity has higher risk of malignancy and progression to invasive carcinoma. However, the postsurgical prognosis for a patient with IPNB is good, with no need of adjuvant therapy. We present the case of a patient with biliary septic shock that prompted urgent surgical intervention. Diagnosis was histopathologically confirmed with the surgical specimen, which showed malignant transformation. During follow up, the patient developed peritoneal carcinomatosis and progression of the disease despite chemotherapy.