Periampullary Neuroendocrine (Carcinoid) Tumor in a Patient with Neurofibromatosis Type I
Abstract
Neurofibromatosis type 1 (NF-1) is a genetic disease with autosomal dominant pattern which is associated with a large number of tumors especially of Gastro-Intestinal origin. These tumors have nurogenic or neuroendocrine origin (carcinoid tumors) and cause symptoms due to secretion of somatostatin or local pressure effects. Here we report the case of a patient with Neurofibromatosis Type-1 who had a periampullary Nuroendocrine (Carcinoid) Tumor and presented with upper abdominalpain and mild jaundice. An initial diagnosis of carcinoma of head of pancreas was made based on CT scan and Wipple’s Pancreaticoduodenectomy performed. On histopathology, it was revealed as a neuroendocrine tumor with carcinoid features. Patient recovered well and became symptom free after surgery. This concludes that the pancreaticoduodenectomy
(Whippl’s procedure) is the current standard management in periampullary neuroendocrine tumors.
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Copyright (c) 2021 Abdul Qaiyoume Amini, Naveed Ali Khan, Jawed Ahmad, Amjad Siraj Memon
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