Uijeongbu St. Mary’s Hospital, College of Medicine, The Catholic University of Korea Uijeongbu-si, Kyonggi-do, Republic of Korea
Introduction: Annular pancreas is an uncommon congenital malformation. Pancreatic tissue encircles the second portion of duodenum in which the unusual rotation of the pancreatic ventral bud occurs during embryonic development. Moreover, the combination of an annular pancreas with a pancreatic malignancy is extremely rare. Here, we report a rare case of annular pancreas with malignant intraductal papillary mucinous neoplasm (IPMN).
Case Description/Methods: A 82-year-old man was admitted to our hospital with one-month history of jaundice. The patient had a history of hypertension and diabetes. He had icteric sclera and right upper quadrant area pain with Murphy’s sign. In laboratory test, total bilirubin was 24.84 mg/dl, direct bilirubin 19.44 mg/dl, aspartate aminotransferase 45 IU/L, alanine aminotransferase 76 IU/L, alkaline phosphatase 878 U/L, gamma-glutamic transpeptidase 387 U/L, amylase 105 U/L and lipase 109.33 IU/L. Abdominal CT and MRI with MRCP showed a huge IPMN (6 cm sized multi-locular mass) with high risk stigmata in pancreas head, causing extra hepatic biliary obstruction and coincidentally suspicious annular pancreas. We examined the duodenum using upper endoscopy first. It showed multiple irregular active ulcers from bulb to just above the second portion of duodenum with luminal narrowing. Then, ERCP (endoscopic retrograde cholangiopancreatography) revealed that widened ampullary orifice was discharging much mucin and confirmed that encircling ventral annular pancreatic duct (diameter 7mm) was connected to doral pancreatic duct using guidewire and contrast dyes. It showed long-segmental stricture (length 42mm) at the distal bile duct and a biliary plastic stent was inserted after minimal endoscopic sphincterotomy and biopsies at the bile duct stricture. The biopsy result was negative for malignancy, and the brush cytology was performed in the next ERCP session. The brush cytology demonstrated a cluster of malignant glandular cells with nuclear overlapping, nuclear membrane irregularity and loss of polarity. Because of both old age and poor general condition, the patient did not want any treatment. So we just treated him conservatively. Although the total bilirubin was improved to 6.1 mg/dl about 20 days later, he died 2 months after with pregression of cancer, renal failure and sepsis.
Discussion: In conclusion, the possibility of coexisting pancreatobiliary disease or malignancy should be considered in adult patients with an annular pancreas although it is extremly rare.
