A case of periampullary adenocarcinoma in neurofibromatosis type 1

Neurofibromatosis type 1 (NF-1), also known as von Recklinghausen’s disease, is an autosomal-dominant disorder affecting males and females equally with an incidence of approximately one in 3,000 births (1,2). The classic clinical findings include café-au-lait spots, cutaneous neurofibromas, cognitive impairment, axillary and/or inguinal freckling, Lisch nodules (pigmented hamartomatous nevus of the iris) and bony abnormalities. In addition to these findings, patients with NF-1 also have an increased incidence of both benign and malignant neoplasms with the most common sites including the nervous system, skin, muscles and gastrointestinal tract