<prompt> Periampullary region carcinoids are characteristically seen in:Multiple Endocrine Neoplasia 1Von Hippel Lindau diseaseVon Recklinghausens diseaseCri du chat syndrome  <response>: Von Recklinghausens disease patients develop an upper GI carcinoid tumor, characteristically in the periampullary region 54. They contain somatostatin immunocytochemically. Reference: Harrisons Principles of Internal Medicine, 18th Edition, Page 3060 