Intraductal papillary mucinous neoplasms (IPMN) are mucin producing cystic neoplasms of the pancreas. IPMN can rarely present with atypical manifestation such as fistulization into neighboring organs and anatomic structures. We present two cases of IPMN presenting with pancreatico-biliary and pancreatico-duodenal fistulizations.
In our unit, two patients both presenting with signs and symptoms of obstructive Jaundice and chronic pancreatitis, were seen and evaluated. Both patients had weight loss, abdominal pain, jaundice and elevated Ca19, 9 levels. In both cases, a multicyctic mass was found in the pancreatic head. Patients were evaluated with abdominal CT, ERCP and EUS. During work up with these modalities, we encountered pancreaticobiliary and pancreaticoduodenal fistulizations. Fish mouth appearance of the papilla and thick mucous discharge from the papilla along with biliary ductal dilatations were also observed in both cases on ERCP. In the case with duodenal fistulization, we noticed a hole in the duodenum which was an atypical finding without much impact on the clinical course. However, the patient with pancreaticobiliary fistula had obstructive jaundice with repeated episodes of cholangitis due to continuous thick mucous plugging of the common bile duct. Stents were put into the ducts for decompression and drainage on several occasions to relieve obstructive jaundice. Eventually, both patients underwent Whipple procedure.
Histopathological evaluation of the both specimens revealed IPMN with high grade dysplasia . Both fistulas were identified on the specimens .Site of fistulization did not demonstrate any invasion of cancer.
These cases highlight that IPMN can rarely present with fistulas. IPMN can lead to pancreatitis and fistula formation without invasion of the cancer to the surrounding organs. Fistula formation without cancer spread could be linked to a combination of high pressure in the pancreatic duct and ongoing chronic inflammation. Detection of atypical fistulas between the pancreas and the surrounding organs should trigger suspicion and more aggressive approach for detection and treatment of IPMN.
Program Number: P409