Von Recklinghausen neurofibromatosis with carcinoid tumor and submucous leiomyomas of the duodenum.

We report on a 63-year-old man with von Recklinghausen neurofibromatosis who developed a carcinoid tumor in the periampullary region and multiple small-sized leiomyomas in the duodenal wall. A high prevalence of gastrointestinal carcinoids has previously been recognized in patients with von Recklinghausen neurofibromatosis, the commonest site of origin being the duodenum or the periampullary region. The association of both conditions seems to be more than casual. Abnormalities in the development of the complex of von Campenhout or high circulating levels of nerve growth factor have been the mechanisms postulated to account for this association.