Periampullary region carcinoids are characteristically seen in:: (A) Multiple Endocrine Neoplasia 1, (B) Von Hippel Lindau disease, (C) Von Recklinghausen's disease, (D) Cri du chat syndrome
Answer is C. 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