Nonfunctioning pancreatic neuroendocrine tumors presenting as pancreatitis: report of four cases

The presentation of pancreatic adenocarcinoma as acute or chronic pancreatitis has been well documented; however, there has been only one previous report of either functioning or nonfunctioning pancreatic neuroendocrine tumors associated with pancreatitis. At the Medical University of South Carolina in Charleston, from March 1982 through September 1987, we have managed four patients with nonfunctioning pancreatic islet cell tumors or carcinoids, which presented with attacks of pancreatitis. Three of the patients had recurrent bouts of upper abdominal and lower dorsal back pain with elevation of the serum amylase. One patient presented initially with acute upper abdominal pain and elevation of the serum amylase. Each patient had an endoscopic retrograde cholangeography pancreatography (ERCP) pattern involving the pancreatic duct which was characterized by diffuse dilatation proximal to the site of obstruction. One of the four had a tumor blush on splanchnic angiography. Each patient had CT evidence of a mass in the head of the pancreas; however, one of the four was found to have diffuse involvement of the entire gland at operation. Surgical therapy varied: (a) local excision of the ampullary area with re-anastomosis of the pancreatic duct to the duodenum and choledochoduodenostomy; (b) bypass with cholecystoduodenostomy and caudal pancreaticojejunostomy; (e) total pancreatectomy; or (d) bypass with a Roux-en-Y cholecystojejunostomy and gastrojejunostomy. The choice of the procedure was based on the patient's condition and operative findings.