Carcinoid tumors of the ampulla of Vater

Periampullary carcinoid tumors are rare; only 73 have previously been reported in the international literature. We reviewed the records of four patients with carcinoid tumor at Vater's ampulla. The findings were compared to a recent review of the previously reported 73 patients. The two groups were similar with respect to male predominance, presenting symptoms, infrequent preoperative diagnosis, tumor size, and rate of metastasis. The present study patients were older by 20 years, and none of the patients in this group had von Recklinghausen's disease, despite a 25 per cent incidence in the review group. The follow-up in the present study is too short to make conclusions, but the 5-year survival calculated in the review group was 90 per cent. Periampullary carcinoids act differently clinically from the midgut type. In particular, tumor size appears to have no correlation to metastatic potential, as in midgut carcinoids. If carcinoid syndrome does develop, as a foregut carcinoid it may have a "variant" type syndrome.