A Rare Cause of Melaena-Duodenal Gangliocytic Paraganglioma

Carmencita Esquivel; Suresh Navadgi; Greg Otto; Rebecca Thomas

doi:10.4236/ss.2012.34045

Gangliocytic Paraganglioma of the Duodenum: Case Report

Tumori Journal ◽

10.1177/030089168707300419 ◽

1987 ◽

Vol 73 (4) ◽

pp. 425-429 ◽

Cited By ~ 3

Author(s):

Stefania Dante ◽

Giuseppe Viale ◽

Paolo Dalla Palma

Keyword(s):

Case Report ◽

Ganglion Cells ◽

Neuron Specific Enolase ◽

Gangliocytic Paraganglioma ◽

Spindle Cells ◽

S 100 ◽

Neurofilament 200

A case of gangliocytic paraganglioma of the second portion of the duodenal loop is presented. The tumor was polypoid and, histologically, composed of mature ganglion cells, spindle cells and epithelial-like cells. Immunocytochemical examination demonstrated the presence of neurofilament 200 K and S-100 protein only in the first two types of cells; all the cells were positive for neuron-specific enolase. The reaction for cytokeratin was negative in all neoplastic components. According to morphologic and immunocytochemical findings, we suggest a hamartomatous nature of this entity.

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Neurofibromatosis-1 and Ampullary Gangliocytic Paraganglioma Causing Biliary and Pancreatic Obstruction

International Journal of Gastrointestinal Cancer ◽

10.1385/ijgc:29:2:093 ◽

2001 ◽

Vol 29 (2) ◽

pp. 093-098 ◽

Cited By ~ 18

Author(s):

Laura Castoldi ◽

Paolo De Rai ◽

Aldo Marini ◽

Stefano Ferrero ◽

Valeria Midolo De Luca ◽

...

Keyword(s):

Neurofibromatosis 1 ◽

Gangliocytic Paraganglioma

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

Case Reports in Surgery ◽

10.1155/2018/6292789 ◽

2018 ◽

Vol 2018 ◽

pp. 1-4 ◽

Cited By ~ 2

Author(s):

Laurie Adams ◽

Theodore M. Friedman ◽

Timothy R. Shaver ◽

George Younan

Keyword(s):

Radical Resection ◽

Major Surgery ◽

Benign Tumors ◽

Neuroendocrine Neoplasms ◽

Gangliocytic Paraganglioma ◽

Appropriate Treatment ◽

Upper Gastrointestinal Bleed ◽

Submucosal Resection ◽

Upper Gastrointestinal ◽

Caucasian Female

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.

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