Periampullary region carcinoids are characteristically seen in:
Von Recklinghausen's 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
Multiple Endocrine Neoplasia 1
Von Hippel Lindau disease
Von Recklinghausen's disease
Cri du chat syndrome