부산대학교 도서관

Neurofibromatosis type 1 (NF-1) is a genetic disease with autosomal dominant pattern which is associatedwith a large number of tumors especially of Gastro-Intestinal origin. These tumors have nurogenic orneuroendocrine origin (carcinoid tumors) and cause symptoms due to secretion of somatostatin or localpressure effects. Here we report the case of a patient with Neurofibromatosis Type-1 who had a periampullaryNuroendocrine (Carcinoid) Tumor and presented with upper abdominalpain and mild jaundice. An initialdiagnosis of carcinoma of head of pancreas was made based on CT scan and Wipple’s Pancreaticoduodenectomyperformed. On histopathology, it was revealed as a neuroendocrine tumor with carcinoid features. Patientrecovered well and became symptom free after surgery. This concludes that the pancreaticoduodenectomy(Whippl’s procedure) is the current standard management in periampullary neuroendocrine tumors.