[Neurofibromatosis (von Recklinghausen disease) and carcinoid tumor in Vater's ampulla]

The localization of the carcinoid tumors of the gastrointestinal tract--as a part of the APUD system--is extremely seldom at the region of ampulla of Vater. A case of congenital neurofibromatosis is reported in a 49-year-old male patient, with a carcinoid tumor of the ampulla of Vater in the background of long-standing, atypical biliary complaints. Periampullary neoplasm of neural-crest origin are very rare, up to this time 73 cases of Vater papilla carcinoid tumors were described. Regarding the development of VP carcinoid in neurofibromatosis, our patient is the 21st reported case in the world literature, and the first one in Hungary. The authors review the literature, giving special attention to the risk of periampullary neoplasms in von Recklinghausen's disease, the need of accurate diagnosis and the correctly selected operative intervention.