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A Rare Case of Thoracoabdominal Paraganglioma: A Case Report and Literature Review

Pheochromocytomas and paragangliomas are rare neuroendocrine tumors. Pheochromocytomas are derived from chromaffin cells of the adrenal medulla, while paragangliomas arise from the extra-adrenal autonomic paraganglia. Paragangliomas can derive from either parasympathetic or sympathetic paraganglia....

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Autores principales: Baptista, Patrícia, Benido Silva, Vânia, Cruz, Ana Rita, Fonseca, Liliana, Palma, Isabel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9837831/
https://www.ncbi.nlm.nih.gov/pubmed/36654613
http://dx.doi.org/10.7759/cureus.32504
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author Baptista, Patrícia
Benido Silva, Vânia
Cruz, Ana Rita
Fonseca, Liliana
Palma, Isabel
author_facet Baptista, Patrícia
Benido Silva, Vânia
Cruz, Ana Rita
Fonseca, Liliana
Palma, Isabel
author_sort Baptista, Patrícia
collection PubMed
description Pheochromocytomas and paragangliomas are rare neuroendocrine tumors. Pheochromocytomas are derived from chromaffin cells of the adrenal medulla, while paragangliomas arise from the extra-adrenal autonomic paraganglia. Paragangliomas can derive from either parasympathetic or sympathetic paraganglia. The majority of parasympathetic ganglia-derived paragangliomas are nonfunctional and symptoms arise from mass effect, while sympathetic paragangliomas are frequently functional and present with symptoms that result from catecholamine hypersecretion. Here, we present the case of a 19-year-old female with hypertension whose biochemical tests revealed elevated plasma and urinary levels of norepinephrine and normetanephrine. Imaging studies showed a left paravertebral mass which was surgically removed. Histopathology confirmed a paraganglioma. Total surgical resection remains the gold-standard treatment and a cure can be achieved; however, all tumors may harbor malignant potential, and a long-term biochemical and imaging follow-up is required in all patients. Screening for genetic germline mutations may be helpful in identifying patients with a higher risk of recurrence or of developing other primary tumors.
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spelling pubmed-98378312023-01-17 A Rare Case of Thoracoabdominal Paraganglioma: A Case Report and Literature Review Baptista, Patrícia Benido Silva, Vânia Cruz, Ana Rita Fonseca, Liliana Palma, Isabel Cureus Endocrinology/Diabetes/Metabolism Pheochromocytomas and paragangliomas are rare neuroendocrine tumors. Pheochromocytomas are derived from chromaffin cells of the adrenal medulla, while paragangliomas arise from the extra-adrenal autonomic paraganglia. Paragangliomas can derive from either parasympathetic or sympathetic paraganglia. The majority of parasympathetic ganglia-derived paragangliomas are nonfunctional and symptoms arise from mass effect, while sympathetic paragangliomas are frequently functional and present with symptoms that result from catecholamine hypersecretion. Here, we present the case of a 19-year-old female with hypertension whose biochemical tests revealed elevated plasma and urinary levels of norepinephrine and normetanephrine. Imaging studies showed a left paravertebral mass which was surgically removed. Histopathology confirmed a paraganglioma. Total surgical resection remains the gold-standard treatment and a cure can be achieved; however, all tumors may harbor malignant potential, and a long-term biochemical and imaging follow-up is required in all patients. Screening for genetic germline mutations may be helpful in identifying patients with a higher risk of recurrence or of developing other primary tumors. Cureus 2022-12-14 /pmc/articles/PMC9837831/ /pubmed/36654613 http://dx.doi.org/10.7759/cureus.32504 Text en Copyright © 2022, Baptista et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Endocrinology/Diabetes/Metabolism
Baptista, Patrícia
Benido Silva, Vânia
Cruz, Ana Rita
Fonseca, Liliana
Palma, Isabel
A Rare Case of Thoracoabdominal Paraganglioma: A Case Report and Literature Review
title A Rare Case of Thoracoabdominal Paraganglioma: A Case Report and Literature Review
title_full A Rare Case of Thoracoabdominal Paraganglioma: A Case Report and Literature Review
title_fullStr A Rare Case of Thoracoabdominal Paraganglioma: A Case Report and Literature Review
title_full_unstemmed A Rare Case of Thoracoabdominal Paraganglioma: A Case Report and Literature Review
title_short A Rare Case of Thoracoabdominal Paraganglioma: A Case Report and Literature Review
title_sort rare case of thoracoabdominal paraganglioma: a case report and literature review
topic Endocrinology/Diabetes/Metabolism
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9837831/
https://www.ncbi.nlm.nih.gov/pubmed/36654613
http://dx.doi.org/10.7759/cureus.32504
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