Ganglioneuroma in the papilla of Vater with neurofibromatosis type 1: report of a case

Ganglioneuromas (GNs) are rare benign tumors and their association with neurofibromatosis type 1 (NF-1) is especially uncommon. We report in this article the case of a young woman, subjected to diagnostic work-up because of abdominal pain. Endoscopy and histology revealed not only a GN in the papill...

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Bibliographic Details
Published in:Surgery today (Tokyo, Japan) Japan), 2013-06, Vol.43 (6), p.675-677
Main Authors: Papp, Róbert, Baracs, József, Papp, András, Tornóczki, Tamás, Vincze, Áron, Horváth, Örs Péter, Kelemen, Dezső
Format: Article
Language:English
Subjects:
Adult
Ampulla of Vater - surgery
Case Report
Common Bile Duct Neoplasms - diagnosis
Common Bile Duct Neoplasms - etiology
Common Bile Duct Neoplasms - pathology
Common Bile Duct Neoplasms - surgery
Female
Ganglioneuroma - diagnosis
Ganglioneuroma - etiology
Ganglioneuroma - pathology
Ganglioneuroma - surgery
Humans
Medicine
Medicine & Public Health
Neurofibromatosis 1 - complications
Pancreaticoduodenectomy
Surgery
Surgical Oncology
Treatment Outcome
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Summary:Ganglioneuromas (GNs) are rare benign tumors and their association with neurofibromatosis type 1 (NF-1) is especially uncommon. We report in this article the case of a young woman, subjected to diagnostic work-up because of abdominal pain. Endoscopy and histology revealed not only a GN in the papilla of Vater, but also NF-1. Because of the size and macroscopic features of the lesion, we performed pancreatoduodenectomy, from which she recovered uneventfully. Histological examination of the resected tumor confirmed a diagnosis of GN.
ISSN:0941-1291
1436-2813
DOI:10.1007/s00595-012-0367-6