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Pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review

BACKGROUND: Gangliocytic paraganglioma (GP) is a rare neuroendocrine neoplasm, which occurs mostly in the periampullary portion of the duodenum; the majority of the reported cases of duodenal GP has been of benign nature with a low incidence of regional lymph node metastasis. GP arising from the pan...

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Autores principales: Nonaka, Keisuke, Matsuda, Yoko, Okaniwa, Akira, Kasajima, Atsuko, Sasano, Hironobu, Arai, Tomio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5540335/
https://www.ncbi.nlm.nih.gov/pubmed/28764742
http://dx.doi.org/10.1186/s13000-017-0648-x
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author Nonaka, Keisuke
Matsuda, Yoko
Okaniwa, Akira
Kasajima, Atsuko
Sasano, Hironobu
Arai, Tomio
author_facet Nonaka, Keisuke
Matsuda, Yoko
Okaniwa, Akira
Kasajima, Atsuko
Sasano, Hironobu
Arai, Tomio
author_sort Nonaka, Keisuke
collection PubMed
description BACKGROUND: Gangliocytic paraganglioma (GP) is a rare neuroendocrine neoplasm, which occurs mostly in the periampullary portion of the duodenum; the majority of the reported cases of duodenal GP has been of benign nature with a low incidence of regional lymph node metastasis. GP arising from the pancreas is extremely rare. To date, only three cases have been reported and its clinical characteristics are largely unknown. CASE PRESENTATION: A nodule located in the pancreatic head was incidentally detected in an asymptomatic 68-year-old woman. Computed tomography revealed 18-, 8-, and 12-mm masses in the pancreatic head, the pancreatic tail, and the left adrenal gland, respectively. Subsequent genetic examination revealed an absence of mutations in the MEN1 and VHL genes. Macroscopically, the tumor located in the pancreatic head was 22 mm in size and displayed an ill-circumscribed margin along with yellowish-white color. Microscopically, it was composed of three cell components: epithelioid cells, ganglion-like cells, and spindle cells, which led to the diagnosis of GP. The tumor was accompanied by a peripancreatic lymph node metastasis. The tumor in the pancreatic tail was histologically classified as a neuroendocrine tumor (NET) G1 (grade 1, WHO 2010), whereas the tumor in the left adrenal gland was identified as an adrenocortical adenoma. The patient was disease-free at the 12-month follow-up examination. CONCLUSIONS: Pancreatic GP is associated with a higher incidence of metastasis and larger tumor size than duodenal GPs, suggesting that the primary organ of GP is an important prognostic factor.
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spelling pubmed-55403352017-08-03 Pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review Nonaka, Keisuke Matsuda, Yoko Okaniwa, Akira Kasajima, Atsuko Sasano, Hironobu Arai, Tomio Diagn Pathol Case Report BACKGROUND: Gangliocytic paraganglioma (GP) is a rare neuroendocrine neoplasm, which occurs mostly in the periampullary portion of the duodenum; the majority of the reported cases of duodenal GP has been of benign nature with a low incidence of regional lymph node metastasis. GP arising from the pancreas is extremely rare. To date, only three cases have been reported and its clinical characteristics are largely unknown. CASE PRESENTATION: A nodule located in the pancreatic head was incidentally detected in an asymptomatic 68-year-old woman. Computed tomography revealed 18-, 8-, and 12-mm masses in the pancreatic head, the pancreatic tail, and the left adrenal gland, respectively. Subsequent genetic examination revealed an absence of mutations in the MEN1 and VHL genes. Macroscopically, the tumor located in the pancreatic head was 22 mm in size and displayed an ill-circumscribed margin along with yellowish-white color. Microscopically, it was composed of three cell components: epithelioid cells, ganglion-like cells, and spindle cells, which led to the diagnosis of GP. The tumor was accompanied by a peripancreatic lymph node metastasis. The tumor in the pancreatic tail was histologically classified as a neuroendocrine tumor (NET) G1 (grade 1, WHO 2010), whereas the tumor in the left adrenal gland was identified as an adrenocortical adenoma. The patient was disease-free at the 12-month follow-up examination. CONCLUSIONS: Pancreatic GP is associated with a higher incidence of metastasis and larger tumor size than duodenal GPs, suggesting that the primary organ of GP is an important prognostic factor. BioMed Central 2017-08-02 /pmc/articles/PMC5540335/ /pubmed/28764742 http://dx.doi.org/10.1186/s13000-017-0648-x Text en © The Author(s). 2017 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Nonaka, Keisuke
Matsuda, Yoko
Okaniwa, Akira
Kasajima, Atsuko
Sasano, Hironobu
Arai, Tomio
Pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review
title Pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review
title_full Pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review
title_fullStr Pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review
title_full_unstemmed Pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review
title_short Pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review
title_sort pancreatic gangliocytic paraganglioma harboring lymph node metastasis: a case report and literature review
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5540335/
https://www.ncbi.nlm.nih.gov/pubmed/28764742
http://dx.doi.org/10.1186/s13000-017-0648-x
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