A Rare Case of Acute Cholangitis in a Young Male with Tropical Chronic Pancreatitis-Induced Pancreatic Adenocarcinoma with Metastasis to the Liver

Abstract

Tropical chronic pancreatitis (TCP) is a rare and poorly understood variant of chronic pancreatitis. It is typically diagnosed in young, nonalcoholic patients in tropical regions such as southern Asia, often presenting with abdominal pain, and leading to several complications, including diabetes, malnutrition, and pancreatic adenocarcinoma. Though the link between chronic pancreatitis and cancer is established, the risk for malignancy is highest in those with rare causes of chronic pancreatitis. We present a case of TCP-induced pancreatic adenocarcinoma complicated by acute cholangitis. A 34 year-old Bangladeshi male with TCP complicated by diabetes and stage IV pancreatic adenocarcinoma with liver metastasis, s/p whipple procedure, and 3 cycles of adjuvant Gemcitabine and Abraxane presented with acute worsening of chronic abdominal pain. Labs were remarkable for elevated liver enzymes and a marked increase in CA 19-9. CT Abdomen/Pelvis revealed progression of metastatic disease in the liver and upper abdomen, including the peritoneum and anterior abdominal wall. His pain persisted despite EUS-guided celiac plexus block, but became acutely worse on hospital day 15 when he became febrile, jaundiced, and with blood cultures growing E. Coli, Klebsiella, and Aeromonas. Due to concern for acute cholangitis secondary to stenosis of hepaticojejunostomy, he was taken for ERCP. The biliary system was not successfully identified, so he then underwent IR-guided placement of a biliary drain via percutaneous transhepatic cholangiography, which showed central biliary stenosis and severe bilateral ductal dilation. He was subsequently placed in hospice due to his poor prognosis. The etiology of TCP is multifactorial, but also associated with a mutation of the SPINK1 gene, which causes parenchymal damage by lowering the threshold for trypsin activation. Characteristic radiographic findings include a dilated pancreatic duct with large intra-ductal calculi, pancreatic calcifications, and pancreatic atrophy. Data suggests that only 5% of patients with a 20-year history of chronic pancreatitis develop cancer, but 17% of patients with TCP develop malignancy, which is significantly higher than the near 0% risk of malignancy in those with chronic alcoholic pancreatitis. TCP is a rare disease that potentiates many long-term complications that can significantly increase mortality in an otherwise young and healthy population.1273_A.tif Figure 1: Marked dilation of pancreatic duct measuring up to 18 mm (blue arrow) that terminates at the pancreatic head due to a large calcification (red arrow).1273_B.tif Figure 2: Fine needle aspiration of mass at pancreatic head (blue arrow) and dilated CBD measuring up to 13mm (red arrow).1273_C.tif Figure 3: Multiple hepatic metastatic lesions (blue arrows) and invasion of the pancreatic head mass into the anterior abdominal wall (red arrow).