2017 Volume 59 Issue 12 Pages 2732-2739
A 40-year-old female was found to have pancreatico-biliary maljunction on abdominal computed tomography. She has neurofibromatosis type 1 (NF1) with a family history for NF1. She has had an elevated alkaline phosphatase level in the serum and was eventually diagnosed as having osteomalacia at 40 years of age. When endoscopic retrograde cholangiopancreatography was performed to confirm the diagnosis of pancreatico-biliary maljunction, a white raised lesion with a diameter of 3mm was observed in the papilla of Vater. Pathologic examination of the biopsy specimen of the lesion raised the suspicion of neuroendocrine tumor (NET). Pancreatoduodenectomy was performed and the final diagnosis was NET G1. Patients with a gastrointestinal tumor associated with NF1 are usually symptomatic and there have only been a few reports on asymptomatic patients with this condition. Nevertheless, our case suggests that asymptomatic patients with NF1 should undergo gastrointestinal screening.
