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Pheochromocytoma and Paraganglioma: A Review of Diagnosis, Management and Treatment of Rare Causes of Hypertension

Pheochromocytomas (PHEO) and paragangliomas (PGL) are rare tumors originated in cells derived from the neural crest. The first ones are located in the adrenal medulla, and the second ones in the sympathetic and parasympathetic nervous system. These kind of tumors may secrete excess catecholamines, i...

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Autores principales: Cerqueira, Ana, Seco, Tiago, Costa, Ana, Tavares, Maria, Cotter, Jorge
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7273359/
https://www.ncbi.nlm.nih.gov/pubmed/32523826
http://dx.doi.org/10.7759/cureus.7969
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author Cerqueira, Ana
Seco, Tiago
Costa, Ana
Tavares, Maria
Cotter, Jorge
author_facet Cerqueira, Ana
Seco, Tiago
Costa, Ana
Tavares, Maria
Cotter, Jorge
author_sort Cerqueira, Ana
collection PubMed
description Pheochromocytomas (PHEO) and paragangliomas (PGL) are rare tumors originated in cells derived from the neural crest. The first ones are located in the adrenal medulla, and the second ones in the sympathetic and parasympathetic nervous system. These kind of tumors may secrete excess catecholamines, including epinephrine, norepinephrine, dopamine and/or their metabolite metanephrine, normetanephrine and 3-methoxytyramine, respectively. Its clinical manifestations depend on the location, the secretory profile and the malignant potential of the tumor. These tumors are frequently benign in their presentation. Some arise in the context of familiar syndromes, accounting for up to one-third of the total of diagnosis. The metastatic form is the most common presentation of the tumors with familiar origin and due to their rarity, their diagnosis and management is often difficult. Over the years, our knowledge and perception of PHEO and PGL has greatly expanded and changed. This review article aims to focus on the genetic, clinical, diagnostic, therapeutic and prognostic approaches, to give the clinician knowledge of the most recent updates regarding these themes.
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spelling pubmed-72733592020-06-09 Pheochromocytoma and Paraganglioma: A Review of Diagnosis, Management and Treatment of Rare Causes of Hypertension Cerqueira, Ana Seco, Tiago Costa, Ana Tavares, Maria Cotter, Jorge Cureus Cardiology Pheochromocytomas (PHEO) and paragangliomas (PGL) are rare tumors originated in cells derived from the neural crest. The first ones are located in the adrenal medulla, and the second ones in the sympathetic and parasympathetic nervous system. These kind of tumors may secrete excess catecholamines, including epinephrine, norepinephrine, dopamine and/or their metabolite metanephrine, normetanephrine and 3-methoxytyramine, respectively. Its clinical manifestations depend on the location, the secretory profile and the malignant potential of the tumor. These tumors are frequently benign in their presentation. Some arise in the context of familiar syndromes, accounting for up to one-third of the total of diagnosis. The metastatic form is the most common presentation of the tumors with familiar origin and due to their rarity, their diagnosis and management is often difficult. Over the years, our knowledge and perception of PHEO and PGL has greatly expanded and changed. This review article aims to focus on the genetic, clinical, diagnostic, therapeutic and prognostic approaches, to give the clinician knowledge of the most recent updates regarding these themes. Cureus 2020-05-05 /pmc/articles/PMC7273359/ /pubmed/32523826 http://dx.doi.org/10.7759/cureus.7969 Text en Copyright © 2020, Cerqueira et al. http://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 Cardiology
Cerqueira, Ana
Seco, Tiago
Costa, Ana
Tavares, Maria
Cotter, Jorge
Pheochromocytoma and Paraganglioma: A Review of Diagnosis, Management and Treatment of Rare Causes of Hypertension
title Pheochromocytoma and Paraganglioma: A Review of Diagnosis, Management and Treatment of Rare Causes of Hypertension
title_full Pheochromocytoma and Paraganglioma: A Review of Diagnosis, Management and Treatment of Rare Causes of Hypertension
title_fullStr Pheochromocytoma and Paraganglioma: A Review of Diagnosis, Management and Treatment of Rare Causes of Hypertension
title_full_unstemmed Pheochromocytoma and Paraganglioma: A Review of Diagnosis, Management and Treatment of Rare Causes of Hypertension
title_short Pheochromocytoma and Paraganglioma: A Review of Diagnosis, Management and Treatment of Rare Causes of Hypertension
title_sort pheochromocytoma and paraganglioma: a review of diagnosis, management and treatment of rare causes of hypertension
topic Cardiology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7273359/
https://www.ncbi.nlm.nih.gov/pubmed/32523826
http://dx.doi.org/10.7759/cureus.7969
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