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BACKGROUND: The association of pancreatitis with neuroendocrine tumors of the pancreas is uncommon, whereas its association with exocrine pancreatic cancer is well recognized. Since the latter, but not the islet cell tumor, is thought to originate within the ductal system, it is not surprising that pancreatitis is less likely to result from the islet cell tumor. The senior author has recently noted that the first recognizable indication of an islet cell carcinoma may be one or more discrete attacks of acute pancreatitis. METHODS AND RESULTS: Acute pancreatitis, resulting from an islet cell tumor, has been observed in five patients, in one of whom it had become chronic. In four of the patients, the tumor was malignant. A review of the English language literature reveals 14 such patients with pancreatitis, and 2 others have been noted in other languages (a total of 21 patients). The tumor was malignant in 15 of the 21 patients (71%). Although the development of pancreatitis was found to result from the obstruction of the duct in most patients, at least three seemed unrelated. In 12 of 21 cases (57%), an acute attack of pancreatitis, usually recurrent, was the initial syndrome, in contradistinction to pancreatitis resulting from exocrine tumors, which has usually been chronic in nature. CONCLUSION: Pancreatitis, particularly acute pancreatitis, may result from an islet cell tumor. Although unusual, its occurrence may be the first signal of the presence of the tumor. In our experience, obstruction of the pancreatic duct by the islet cell carcinoma appears to be the important etiologic factor. Am J Surg. 1996;171:562-564.

PURPOSE: The purpose of this work was to evaluate the proposed usefulness of a standard meal-stimulated gastrin provocative test in: (1) distinguishing Zollinger-Ellison syndrom (ZES) from antral syndromes; (2) localizing duodenal gastrinomas; or (3) suggesting that patients with multiple endocrine neoplasia type I (MEN-I) may have an increased incidence of antral syndromes. PATIENTS AND METHODS: Seventy-four consecutive patients with ZES referred to the National Institutes of Health were studied prospectively. The extent and location of gastrinomas, acid secretory studies, and the presence or absence of MEN-I were determined and correlated with the results of the gastrin response to standard meal provocative testing. RESULTS: For patients with fasting serum gastrin levels less than 1,000 pg/mL (n = 43), only 44% had a less than 50% increase over the premeal value, which is reported to be the typical response in ZES, and 40% had a 50% to 99% increase. Furthermore, 16% had a 100% or greater increase, 9% a 150% or greater increase, and 5% a 200% or greater increase, which overlaps with values reported to be characteristic of 98%, 92%, and 46% of patients with antral syndromes. Results did not differ for patients with or without MEN-I, depend on the extent of the gastrinoma (duodenal versus pancreatic gastrinomas), the presence of previous gastric surgery or type of gastric surgery, or for patients with fasting serum gastrin concentrations greater than or equal to 1,000 pg/mL or less than 1,000 pg/mL. Studies of four patients before and after resection of the gastrinoma, who prior to surgery had a greater than 100% increase in gastrin secretion after the meal, demonstrated that all patients had a less than 100% increase postoperatively even though no gastric resection was done. CONCLUSIONS: Approximately half of the patients with ZES have a greater than 50% increase in serum gastrin concentration following a standard test meal and one fifth have a 100% or greater increase. Therefore, they cannot be distinguished on this basis from patients with antral syndromes. The increased serum gastrin level after the meal in these patients with ZES appears to be due to the gastrinoma. Furthermore, the current study provides no evidence for the proposals that antral syndromes are more common in patients with MEN-I, that gastric surgery affects the meal response in patients with gastrinomas, or that the meal test is useful in localizing duodenal gastrinomas., Zollinger-Ellison syndrome (ZES) is a disorder characterized by extreme hyperacidity of the stomach, severe peptic ulcer disease and tumors of the pancreas. It is usually diagnosed on the basis of a fasting blood test which shows an elevated level of gastrin, a hormone which stimulates the secretion of gastric acid in the stomach. However, elevated gastrin levels may also be indicative of antral syndrome, or hyperactivity of antral cells (located in the antrum of the stomach). It is thought that after eating a meal, patients with ZES experience little or no change in their gastrin level, while those with antral syndrome show an exaggerated increase in gastrin (normally distension of the antrum causes an increased release of gastrin). Previous studies have suggested that a provocative meal test can help to distinguish between these two conditions and identify the location of a gastrinoma (gastrin tumor). It has been suggested that patients with multiple endocrine neoplasia type I (MEN-I), a genetic disorder associated with endocrine glands tumors, have a high rate of antral syndrome and gastrinomas. A patient's response to the meal test may be different if he or she has undergone stomach surgery. An evaluation of the usefulness of the provocative meal test was conducted to distinguish ZES from antral syndromes, to determine the location of gastrinomas and to suggest that patients who have MEN-I also have a higher frequency of antral syndrome. Seventy-four patients with ZES were given standard provocative meal testing. The results indicate that the provocative meal test was not reliable in differentiating ZES from antral syndrome, and there was no indication that patients with MEN-I have a higher incidence of antral syndrome. In addition, the meal test was not found to be useful in locating gastrinomas. No conclusion was drawn about whether the patient's response to the test was affected by prior surgery.