Abstract
Von Recklinghausen’s disease (VRD) is a hereditary disease that occurs because of mutation in NF1 gene located on chromosome 17. About 25% of VRD patients are reported to suffer from gastrointestinal lesions varying from hyperplasia of the gut neural tissue, endocrine tumors of the duodenum and the periampullary region, and gastrointestinal stromal tumors. Herein, we describe a case of ampullary neuroendocrine tumor with unique histology and immunohistochemistry in a 35-year-old male patient with VRD, detailed review of literature.
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