Randomized controlled trial of standardized meal stimulation for the detection of enteropancreatic endocrine tumours in patients with multiple endocrine neoplasia type 1

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Abstract Background Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by the occurrence of hyperparathyroidism (approximately 100 per cent), and pituitary (approximately 40 per cent) and enteropancreatic endocrine (approximately 70 per cent) tumours. It has been suggested previously that serum pancreatic polypeptide and serum gastrin after standardized meal test stimulation are useful indicators for early identification of enteropancreatic tumours in MEN1. Therefore the usefulness of the meal test was evaluated in a screening programme of MEN1 mutation carriers. Methods In a prospective, controlled trial between September 1997 and December 1995, 55 standardized meal stimulation tests (563 kcal) were performed in 13 MEN1 mutation carriers with no evidence of enteropancreatic tumours (group 1); ten patients with MEN1 with histologically, biochemically and radiologically evident enteropancreatic tumours (group 2); and in 28 healthy adults for control (group 3). Genetic analysis was done by single-strand conformation analysis and direct sequencing. Serum pancreatic polypeptide and serum gastrin levels were measured by radioimmunoassay (Ideon, Sweden). Data were analysed using analysis of variance (Kruskal–Wallis test, 2 d.f.) and Mann–Whitney test. Results Median (range) basal serum pancreatic polypeptide levels were 214 (38–897) pg ml−1 in group 1, 126 (13–263) pg ml−1 in group 2 and 73 (13–357) pg ml−1 in group 3. Median (range) serum gastrin levels were 103 (37–391), 551 (25–4080) and 65 (17–198) pg ml−1 respectively, but gastrin levels in group 2 were strongly influenced by patients with known gastrinoma. Levels of both variables increased after meal stimulation in all groups. However, there was no significant difference between the groups after stimulation (P > 0·05). Conclusion Standardized meal stimulation test does not reliably indicate the presence of enteropancreatic endocrine tumours in patients with MEN1. It is not therefore used in the authors' routine screening programme for patients with MEN1.