Duodenal Gangliocytic Paraganglioma Requiring a Pancreaticoduodenectomy: A Case Report and Review of the Literature

Introduction. Duodenal gangliocytic paragangliomas (GPs) are a subclass of duodenal neuroendocrine neoplasms and are exceedingly rare. They have been associated with an indolent behavior; however, they can rarely metastasize. Radical resection like a pancreaticoduodenectomy is sometimes indicated. W...

Full description

Saved in:
Bibliographic Details
Main Authors: Laurie Adams, Theodore M. Friedman, Timothy R. Shaver, George Younan
Format: Article
Language:English
Published: Wiley 2018-01-01
Series:Case Reports in Surgery
Online Access:http://dx.doi.org/10.1155/2018/6292789
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1832549238297329664
author Laurie Adams
Theodore M. Friedman
Timothy R. Shaver
George Younan
author_facet Laurie Adams
Theodore M. Friedman
Timothy R. Shaver
George Younan
author_sort Laurie Adams
collection DOAJ
description Introduction. Duodenal gangliocytic paragangliomas (GPs) are a subclass of duodenal neuroendocrine neoplasms and are exceedingly rare. They have been associated with an indolent behavior; however, they can rarely metastasize. Radical resection like a pancreaticoduodenectomy is sometimes indicated. We hereby present a case requiring major surgery and perform a literature search about this disease. Presentation of Case. A 49-year-old Caucasian female, who presented with an upper gastrointestinal bleed, was found to have a hypodense mass in the second/third portion of the duodenum. A biopsy of the mass during upper endoscopy was inconclusive. A pancreaticoduodenectomy was recommended based on the high suspicion for a duodenal adenocarcinoma and was performed successfully. Her final pathology revealed a duodenal gangliocytic paraganglioma. Discussion. The majority of duodenal GPs present as gastrointestinal bleeds while others less commonly present with anemia, abdominal pain, duodenal obstructive symptoms, pancreatitis, or abnormal incidental findings on axial abdominal imaging. Duodenal GPs were initially viewed as benign tumors of the duodenum; however, there have been increasing incidence reports of hematogenous and lymphatic metastasis. Appropriate treatment of duodenal GPs is still controversial and ranges from local endoscopic submucosal resection to major radical surgery. Conclusion. Duodenal GPs are very rare tumors of the second portion of the duodenum presenting with upper gastrointestinal bleeding and local symptoms of surrounding organs. Local or radical resection is usually recommended to prevent bleeding and the minor risk of metastatic spread.
format Article
id doaj-art-71edd530b45c4d09832a87809c4b609a
institution Kabale University
issn 2090-6900
2090-6919
language English
publishDate 2018-01-01
publisher Wiley
record_format Article
series Case Reports in Surgery
spelling doaj-art-71edd530b45c4d09832a87809c4b609a2025-02-03T06:11:45ZengWileyCase Reports in Surgery2090-69002090-69192018-01-01201810.1155/2018/62927896292789Duodenal Gangliocytic Paraganglioma Requiring a Pancreaticoduodenectomy: A Case Report and Review of the LiteratureLaurie Adams0Theodore M. Friedman1Timothy R. Shaver2George Younan3Department of Surgery, Inova Fairfax Hospital, Fairfax, VA, USADepartment of Pathology, Inova Fair Oaks Hospital, Fairfax, VA, USADivision of Hepato-Pancreato-Biliary Surgery, Virginia Surgery Associates, Fairfax, VA, USADivision of Hepato-Pancreato-Biliary Surgery, Virginia Surgery Associates, Fairfax, VA, USAIntroduction. Duodenal gangliocytic paragangliomas (GPs) are a subclass of duodenal neuroendocrine neoplasms and are exceedingly rare. They have been associated with an indolent behavior; however, they can rarely metastasize. Radical resection like a pancreaticoduodenectomy is sometimes indicated. We hereby present a case requiring major surgery and perform a literature search about this disease. Presentation of Case. A 49-year-old Caucasian female, who presented with an upper gastrointestinal bleed, was found to have a hypodense mass in the second/third portion of the duodenum. A biopsy of the mass during upper endoscopy was inconclusive. A pancreaticoduodenectomy was recommended based on the high suspicion for a duodenal adenocarcinoma and was performed successfully. Her final pathology revealed a duodenal gangliocytic paraganglioma. Discussion. The majority of duodenal GPs present as gastrointestinal bleeds while others less commonly present with anemia, abdominal pain, duodenal obstructive symptoms, pancreatitis, or abnormal incidental findings on axial abdominal imaging. Duodenal GPs were initially viewed as benign tumors of the duodenum; however, there have been increasing incidence reports of hematogenous and lymphatic metastasis. Appropriate treatment of duodenal GPs is still controversial and ranges from local endoscopic submucosal resection to major radical surgery. Conclusion. Duodenal GPs are very rare tumors of the second portion of the duodenum presenting with upper gastrointestinal bleeding and local symptoms of surrounding organs. Local or radical resection is usually recommended to prevent bleeding and the minor risk of metastatic spread.http://dx.doi.org/10.1155/2018/6292789
spellingShingle Laurie Adams
Theodore M. Friedman
Timothy R. Shaver
George Younan
Duodenal Gangliocytic Paraganglioma Requiring a Pancreaticoduodenectomy: A Case Report and Review of the Literature
Case Reports in Surgery
title Duodenal Gangliocytic Paraganglioma Requiring a Pancreaticoduodenectomy: A Case Report and Review of the Literature
title_full Duodenal Gangliocytic Paraganglioma Requiring a Pancreaticoduodenectomy: A Case Report and Review of the Literature
title_fullStr Duodenal Gangliocytic Paraganglioma Requiring a Pancreaticoduodenectomy: A Case Report and Review of the Literature
title_full_unstemmed Duodenal Gangliocytic Paraganglioma Requiring a Pancreaticoduodenectomy: A Case Report and Review of the Literature
title_short Duodenal Gangliocytic Paraganglioma Requiring a Pancreaticoduodenectomy: A Case Report and Review of the Literature
title_sort duodenal gangliocytic paraganglioma requiring a pancreaticoduodenectomy a case report and review of the literature
url http://dx.doi.org/10.1155/2018/6292789
work_keys_str_mv AT laurieadams duodenalgangliocyticparagangliomarequiringapancreaticoduodenectomyacasereportandreviewoftheliterature
AT theodoremfriedman duodenalgangliocyticparagangliomarequiringapancreaticoduodenectomyacasereportandreviewoftheliterature
AT timothyrshaver duodenalgangliocyticparagangliomarequiringapancreaticoduodenectomyacasereportandreviewoftheliterature
AT georgeyounan duodenalgangliocyticparagangliomarequiringapancreaticoduodenectomyacasereportandreviewoftheliterature