Obstructive Jaundice Caused by Metastatic Neuroendocrine Tumor of the Ampulla of Vater in a Young Adult: A Case Report

Introduction: Ampullary neuroendocrine tumors (NETs) are usually diagnosed in the 5th–6th decades of life, and no cases were reported in <20 years of age. We report a rare case, presenting at a very young age, of well-differentiated NET involving the ampulla of Vater with lymph node metastasi...

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Main Authors: Jaafar Alsalman, Abdulaziz Alquorain, Mohammed Alyousef, Omran Aldandan, Ibrahim Alzahrani
Format: Article
Language:English
Published: Karger Publishers 2024-04-01
Series:Case Reports in Gastroenterology
Subjects:
Online Access:https://beta.karger.com/Article/FullText/538260
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author Jaafar Alsalman
Abdulaziz Alquorain
Mohammed Alyousef
Omran Aldandan
Ibrahim Alzahrani
author_facet Jaafar Alsalman
Abdulaziz Alquorain
Mohammed Alyousef
Omran Aldandan
Ibrahim Alzahrani
author_sort Jaafar Alsalman
collection DOAJ
description Introduction: Ampullary neuroendocrine tumors (NETs) are usually diagnosed in the 5th–6th decades of life, and no cases were reported in <20 years of age. We report a rare case, presenting at a very young age, of well-differentiated NET involving the ampulla of Vater with lymph node metastasis. Case Presentation: An 18-year-old man presented with a 3-month history of upper abdominal pain and jaundice. Abdominal ultrasound showed a dilated common bile duct, and endoscopic retrograde cholangiopancreatography revealed two duodenal polypoid lesions, one of them overlying the ampulla of Vater, with an erythematous and ulcerated surface. Histopathological examination confirmed the diagnosis of NET grade 1. Octreotide scan revealed 2 para-aortic lymph nodes with intense radiotracer uptake. The patient had undergone Whipple surgery with para-aortic lymph node dissection. Histopathological examination of the surgical specimens was confirmatory of NET grade 2 and paraganglioma in a few of the dissected lymph nodes. Postoperatively, the patient was kept on monthly intramuscular octreotide. Follow-up gallium-68 DOTATATE is unremarkable apart from an avid left para-aortic lymph node which is showing stability over 12 months of follow-up. Conclusion: This case demonstrates that NETs of the ampulla of Vater can present at a very young age. Radical surgical excision with extended lymph node dissection and postoperative octreotide is associated with better patient outcomes and survival.
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spelling doaj.art-7c1ad31070d344a7a9df80a6f18299b92024-04-18T07:17:48ZengKarger PublishersCase Reports in Gastroenterology1662-06312024-04-0118120421310.1159/000538260538260Obstructive Jaundice Caused by Metastatic Neuroendocrine Tumor of the Ampulla of Vater in a Young Adult: A Case ReportJaafar Alsalman0Abdulaziz Alquorain1Mohammed Alyousef2Omran Aldandan3Ibrahim Alzahrani4Department of Internal Medicine, Division of Gastroenterology, Dammam Medial Complex, Dammam, Saudi ArabiaDepartment of Internal Medicine, Division of Gastroenterology, King Fahd Hospital of the University, Imam Abdulrahman Bin Faisal University, Dammam, Saudi ArabiaDepartment of Pathology, King Fahd Hospital of the University, Imam Abdulrahman Bin Faisal University, Dammam, Saudi ArabiaDepartment of Radiology, King Fahd Hospital of the University, Imam Abdulrahman Bin Faisal University, Dammam, Saudi ArabiaDepartment of Internal Medicine, Division of Gastroenterology, King Fahd Hospital of the University, Imam Abdulrahman Bin Faisal University, Dammam, Saudi ArabiaIntroduction: Ampullary neuroendocrine tumors (NETs) are usually diagnosed in the 5th–6th decades of life, and no cases were reported in <20 years of age. We report a rare case, presenting at a very young age, of well-differentiated NET involving the ampulla of Vater with lymph node metastasis. Case Presentation: An 18-year-old man presented with a 3-month history of upper abdominal pain and jaundice. Abdominal ultrasound showed a dilated common bile duct, and endoscopic retrograde cholangiopancreatography revealed two duodenal polypoid lesions, one of them overlying the ampulla of Vater, with an erythematous and ulcerated surface. Histopathological examination confirmed the diagnosis of NET grade 1. Octreotide scan revealed 2 para-aortic lymph nodes with intense radiotracer uptake. The patient had undergone Whipple surgery with para-aortic lymph node dissection. Histopathological examination of the surgical specimens was confirmatory of NET grade 2 and paraganglioma in a few of the dissected lymph nodes. Postoperatively, the patient was kept on monthly intramuscular octreotide. Follow-up gallium-68 DOTATATE is unremarkable apart from an avid left para-aortic lymph node which is showing stability over 12 months of follow-up. Conclusion: This case demonstrates that NETs of the ampulla of Vater can present at a very young age. Radical surgical excision with extended lymph node dissection and postoperative octreotide is associated with better patient outcomes and survival.https://beta.karger.com/Article/FullText/538260ampulla of vaterlymph node metastasesneuroendocrine tumorpancreaticoduodenectomysmall intestinal
spellingShingle Jaafar Alsalman
Abdulaziz Alquorain
Mohammed Alyousef
Omran Aldandan
Ibrahim Alzahrani
Obstructive Jaundice Caused by Metastatic Neuroendocrine Tumor of the Ampulla of Vater in a Young Adult: A Case Report
Case Reports in Gastroenterology
ampulla of vater
lymph node metastases
neuroendocrine tumor
pancreaticoduodenectomy
small intestinal
title Obstructive Jaundice Caused by Metastatic Neuroendocrine Tumor of the Ampulla of Vater in a Young Adult: A Case Report
title_full Obstructive Jaundice Caused by Metastatic Neuroendocrine Tumor of the Ampulla of Vater in a Young Adult: A Case Report
title_fullStr Obstructive Jaundice Caused by Metastatic Neuroendocrine Tumor of the Ampulla of Vater in a Young Adult: A Case Report
title_full_unstemmed Obstructive Jaundice Caused by Metastatic Neuroendocrine Tumor of the Ampulla of Vater in a Young Adult: A Case Report
title_short Obstructive Jaundice Caused by Metastatic Neuroendocrine Tumor of the Ampulla of Vater in a Young Adult: A Case Report
title_sort obstructive jaundice caused by metastatic neuroendocrine tumor of the ampulla of vater in a young adult a case report
topic ampulla of vater
lymph node metastases
neuroendocrine tumor
pancreaticoduodenectomy
small intestinal
url https://beta.karger.com/Article/FullText/538260
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