Neuroendocrine tumor of the pancreas in a patient with pernicious anemia.

A total of 71 patients has been reported in the English literature to have developed carcinoid tumors in the fundus of the stomach in association with chronic atrophic gastritis secondary to pernicious anemia. The tumors appear to develop from argyrophilic cells in response to hypergastrinemia produced by hyperplastic G cells in the antrum of the stomach. We report a similar patient who, in addition, had a neuroendocrine tumor develop in the neck of the pancreas, which obstructed the pancreatic duct and resulted in severe chronic pancreatitis in the body and tail of the gland. There was an associated splenic vein thrombosis with left-sided portal hypertension. The pancreatic neoplasm was treated by excision, including the caudal 85 per cent of the pancreas and spleen. The gastric carcinoids appeared to diminish in size in response to a octreotide acetate (Sandostatin, Sandoz Pharmaceuticals; Hanover, NJ) administered to promote closure of a low-volume postoperative pancreatic fistula. The gastric lesions may require surgical treatment in the future. We were unable to find other examples of pancreatic neuroendocrine tumor occurring in association with pernicious anemia in a search of the English literature.