Malignant Intraductal Papillary Mucinous Neoplasm With Underlying Annular Pancreas

An 82-year-old man was referred to our hospital with severe jaundice and right upper-quadrant pain. He had a history of hypertension and diabetes. His vital signs were stable. His total bilirubin level was 24.84 mg/dL (normal level, ≤1.2 mg/dL), and his direct bilirubin level was 19.44 mg/dL (normal level, ≤0.3 mg/dL). Abdominal computed tomography and magnetic resonance cholangiopancreatography showed a 6-cm multilocular intraductal papillary mucinous neoplasm in the pancreatic head, causing extrahepatic biliary obstruction suspicious for annular pancreas (Figure A). Endoscopic retrograde cholangiopancreatography showed a narrowed second duodenal portion with multiple ulcers and widened ampullary orifice, discharging mucin (Figure B). The encircling ventral pancreatic duct (diameter, 7 mm) was connected to the dorsal pancreatic duct (Figure C). A long-segmented stricture (length, 42 mm) was seen at the distal common bile duct. Brush cytology was performed at the distal common bile duct stricture after minimal endoscopic sphincterotomy, and a plastic biliary stent was inserted. Cytology showed a cluster of malignant glandular cells (Figure D). The patient and his family wanted only conservative treatment. The total bilirubin level improved to 6.1 mg/dL approximately 20 days later, but he died 2 months after the diagnosis from cancer progression and sepsis. We report a malignant intraductal papillary mucinous neoplasm with underlying annular pancreas.