Periampullary Neuroendocrine (Carcinoid) Tumor in a Patient with Neurofibromatosis Type I
- Resource Type
- article
- Authors
- Abdul Qaiyoume Amini; Naveed Ali Khan; Jawed Ahmad; Amjad Siraj Memon
- Source
- Journal of the Dow University of Health Sciences, Vol 9, Iss 2, Pp 78-80 (2015)
- Subject
- Medicine
- Language
- English
- ISSN
- 1995-2198
2410-2180
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.