Von Recklinghausen's neurofibromatosis associated with duodenal somatostatinoma. A case report and review of the literature.

Somatostatin producing duodenal carcinoids are rare, comprising a mere 2% of small bowel carcinoids and 5-10% of all duodenal tumors. Since the 1st case described by Kaneko in 1979 more than 50 cases have been reported in the world literature. From these reports, it is gradually emerging that duodenal somatostatinomas may show a strong association with von Recklinhausen's neurofibromatosis (VRNF) as a distinct neuroendocrine syndrome. A case of …