1324 Recurrent Pancreatitis Associated With Pancreas Divisum and Rare Solid Pseudopapillary Neoplasm of the Pancreas

Abstract

INTRODUCTION: Pancreas Divisum (PD) is a benign congenital anomaly found in approximately 4-10% of general population. Rare cases of acute pancreatitis associated with PD and pancreatic tumors have been described. While most common causes of pancreatitis are gallstones and alcohol use, uncommon causes such as anatomic/genetic variants and malignancies of the pancreaticobiliary system should be investigated especially in case of recurrent cryptogenic pancreatitis. We present a case of recurrent pancreatitis in a patient with pancreas divisum and rare solid pseudopapillary neoplasm of the pancreas. CASE DESCRIPTION/METHODS: A 65-year old woman with past medical history of psoriatic arthritis on methotrexate therapy and no family history of pancreatitis was evaluated for recurrent pancreatitis. Patient had at least eight episodes of acute pancreatitis from 2011 to 2019. Patient denied any alcohol/tobacco history. Serum triglycerides/calcium were not elevated. Genetic causes were excluded. Imaging ruled out gallstones but showed presence of pancreas divisum. Endoscopic ultrasound (EUS)/Endoscopic retrograde cholangiopancreatography (ERCP) in 2016 was negative for stricture, focal mass or lesions. In April 2017, patient underwent repeat EUS/ERCP with stent placement into the minor papilla for presumed stenosis. However, that resulted in an exacerbation of her symptoms and the stent was subsequently removed. Patient continued to have recurrent acute pancreatitis and underwent repeat EUS in August 2018 which showed an 8 × 8 mm pancreatic genu mass adjacent to the Santorini duct. Biopsy was consistent with rare, slow growing solid pseudopapillary neoplasm (SPN) of the pancreas. Patient underwent pancreaticoduodenectomy in 2019. Pathology confirmed SPN. Patient has had no further episodes of pancreatitis. DISCUSSION: Pancreas Divisum (PD) results from failure of fusion of ventral and dorsal pancreatic ducts during embryogenesis. It has been controversial whether PD by itself causes recurrent pancreatitis. Clearly, in our patient, addressing the PD alone did not resolve her symptoms of recurrent pancreatitis. Sporadic hemorrhage within the SPN and intermittent compression of the Santorini duct was the likely cause of recurrent pancreatitis in our patient. Patients with cryptogenic recurrent pancreatitis should be evaluated periodically for the presence of slow growing tumors that may not be evident on initial imaging and may be the cause of recurrent pancreatitis in such patients.