Grave Clinical Course of Pancreatic Invasive Cancer Developed in the Patients with Branch Duct IPMN: A Report of Three Cases / 대한췌담도학회지

Branch duct intraductal papillary mucinous neoplasms of the pancreas (BD-IPMN) without malignant features rarely developed into invasive cancer. However, invasive cancer is aggressive once an invasive change occurs. We report three cases of invasive cancers which developed in patients with BD-IPMN and they showed grave clinical courses. All patients were diagnosed with BD-IPMN < 3 cm without malignant features on imaging. Invasive cancer was detected at 2.5 years, 3.0 years, and 4.0 years after BD-IPMN detection in each patient. The intervals of invasive cancer and the last follow-up were 9 months, 3 years, and 1.5 years in the three patients, respectively. All patients were diagnosed with locally advanced pancreas invasive cancers and were treated with palliative chemotherapy or conservative management. The patients died at 3 months, 9 months, and 10 months after the diagnosis of invasive cancers, respectively. We report three cases of invasive cancer developed in BD-IPMN patients and followed fatal courses.

A Case of Combined Congenital Hepatic Fibrosis and Biliary Hamartoma Presented with Recurrent Acute Cholangitis / 대한췌담도학회지

Biliary hamartoma and congenital hepatic fibrosis belong to fibrocystic disorders originating from ductal plate malformation. A 66-year-old man who had incidentally been diagnosed with biliary hamartoma two years ago presented to us with recurrent acute cholangitis. In the first episode, he had presented with septic shock and was treated with endoscopic retrograde cholangiopancreatography (ERCP) and cholecystectomy under the diagnosis of acute cholecystitis and cholangitis. However, during a two-month follow-up period, the patient experienced four episodes of acute cholangitis. Because he showed normal ERCP, and biliary hamartoma is usually asymptomatic, a liver biopsy was performed. Pathology revealed combined features of biliary hamartoma and congenital hepatic fibrosis, characterized as periportal fibrosis and intrahepatic ductular dysplasia. During follow-up for the last six months, he had experienced two episodes of acute cholangitis and was treated with antibiotics. A follow-up abdominal CT scan revealed aggravated hepatosplenomegaly compared to that of two years ago. We report a case of combined congenital hepatic fibrosis and biliary hamartoma and a literature review.