Periampullary Neuroendocrine (Carcinoid) Tumor in a Patient with Neurofibromatosis Type I

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...

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Main Authors: Abdul Qaiyoume Amini, Naveed Ali Khan, Jawed Ahmad, Amjad Siraj Memon
Format: Article
Language:English
Published: Dow University of Health Sciences 2015-08-01
Series:Journal of the Dow University of Health Sciences
Online Access:https://www.jduhs.com/index.php/jduhs/article/view/1463
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author Abdul Qaiyoume Amini
Naveed Ali Khan
Jawed Ahmad
Amjad Siraj Memon
author_facet Abdul Qaiyoume Amini
Naveed Ali Khan
Jawed Ahmad
Amjad Siraj Memon
author_sort Abdul Qaiyoume Amini
collection DOAJ
description 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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spelling doaj.art-2e496263b5804cf4827b7dd043bc33362023-05-29T03:10:30ZengDow University of Health SciencesJournal of the Dow University of Health Sciences1995-21982410-21802015-08-0192Periampullary Neuroendocrine (Carcinoid) Tumor in a Patient with Neurofibromatosis Type IAbdul Qaiyoume Amini0Naveed Ali Khan1Jawed Ahmad2Amjad Siraj Memon3Dow University of Health SciencesSurgical Unit V, Dow University of Health Sciences, Civil Hospital, Karachi, Pakistan.Surgical Unit V, Dow University of Health Sciences, Civil Hospital, Karachi, Pakistan.Surgical Unit V, Dow University of Health Sciences, Civil Hospital, Karachi, Pakistan. 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. https://www.jduhs.com/index.php/jduhs/article/view/1463
spellingShingle Abdul Qaiyoume Amini
Naveed Ali Khan
Jawed Ahmad
Amjad Siraj Memon
Periampullary Neuroendocrine (Carcinoid) Tumor in a Patient with Neurofibromatosis Type I
Journal of the Dow University of Health Sciences
title Periampullary Neuroendocrine (Carcinoid) Tumor in a Patient with Neurofibromatosis Type I
title_full Periampullary Neuroendocrine (Carcinoid) Tumor in a Patient with Neurofibromatosis Type I
title_fullStr Periampullary Neuroendocrine (Carcinoid) Tumor in a Patient with Neurofibromatosis Type I
title_full_unstemmed Periampullary Neuroendocrine (Carcinoid) Tumor in a Patient with Neurofibromatosis Type I
title_short Periampullary Neuroendocrine (Carcinoid) Tumor in a Patient with Neurofibromatosis Type I
title_sort periampullary neuroendocrine carcinoid tumor in a patient with neurofibromatosis type i
url https://www.jduhs.com/index.php/jduhs/article/view/1463
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AT naveedalikhan periampullaryneuroendocrinecarcinoidtumorinapatientwithneurofibromatosistypei
AT jawedahmad periampullaryneuroendocrinecarcinoidtumorinapatientwithneurofibromatosistypei
AT amjadsirajmemon periampullaryneuroendocrinecarcinoidtumorinapatientwithneurofibromatosistypei