[Calcitonin-producing duodenal carcinoid in Recklinghausen's neurofibromatosis. Clinical case report and review of the literature].

In a 32-year-old woman presenting with fever, vomiting and right upper abdominal pain a tumor of the papilla of Vater was detected endoscopically and removed surgically. Histologically it was a carcinoid shown immunohistochemically to produce calcitonin. The tumor had metastasized to a lymph node in the hepatoduodenal ligament. Clinically the patient showed typical signs of von Recklinghausen neurofibromatosis. A survey of the literature suggests that carcinoid of the papilla of Vater may be more frequent in patients with neurofibromatosis. Possible relationships between neurofibromatosis and carcinoid are discussed in the light of the pertinent literature.