[Multiple duodenal and ampullary carcinoids and von Recklinghausen's neurofibromatosis]

A clinical case of multiple duodenal carcinoid involving the ampulla in a woman with von Recklinghausen's neurofibromatosis (VRNF) is described. The Authors review the literature of the 20 cases previously reported and discuss the significance of such an association. Carcinoid tumors of the duodenum represent 2-5% of all carcinoid found in the general population. While VRNF-associated duodenal carcinoids are not otherwise distinctive, they tend to be associated to pheochromocytoma. Duodenal carcinoid need to be investigated systematically for their immunocytochemical profile, especially for chromogranins (CG), neuron specific enolase (NSE) and somatostatin.