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Clinical Syndromes and Genetic Screening Strategies of Pheochromocytoma and Paraganglioma
Pheochromocytomas (PCCs) are rare neuroendocrine tumors that originate from chromaffin cells of the adrenal medulla, and paragangliomas (PGLs) are extra-adrenal pheochromocytomas. These can be mainly found in clinical syndromes including multiple endocrine neoplasia (MEN), von Hippel–Lindau (VHL) sy...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Codon Publications
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6308242/ https://www.ncbi.nlm.nih.gov/pubmed/30613466 http://dx.doi.org/10.15586/jkcvhl.2018.113 |
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author | Liu, Peihua Li, Minghao Guan, Xiao Yu, Anze Xiao, Qiao Wang, Cikui Hu, Yixi Zhu, Feizhou Yin, Hongling Yi, Xiaoping Liu, Longfei |
author_facet | Liu, Peihua Li, Minghao Guan, Xiao Yu, Anze Xiao, Qiao Wang, Cikui Hu, Yixi Zhu, Feizhou Yin, Hongling Yi, Xiaoping Liu, Longfei |
author_sort | Liu, Peihua |
collection | PubMed |
description | Pheochromocytomas (PCCs) are rare neuroendocrine tumors that originate from chromaffin cells of the adrenal medulla, and paragangliomas (PGLs) are extra-adrenal pheochromocytomas. These can be mainly found in clinical syndromes including multiple endocrine neoplasia (MEN), von Hippel–Lindau (VHL) syndrome, neurofibromatosis-1 (NF-1) and familial paraganglioma (FPGL). PCCs and PGLs are thought to have the highest degree of heritability among human tumors, and it has been estimated that 60% of the patients have genetic abnormalities. This review provides an overview of the clinical syndrome and the genetic screening strategies of PCCs and PGLs. Comprehensive screening principles and strategies, along with specific screening based on clinical symptoms, biochemical tests and immunohistochemistry, are discussed. |
format | Online Article Text |
id | pubmed-6308242 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Codon Publications |
record_format | MEDLINE/PubMed |
spelling | pubmed-63082422019-01-04 Clinical Syndromes and Genetic Screening Strategies of Pheochromocytoma and Paraganglioma Liu, Peihua Li, Minghao Guan, Xiao Yu, Anze Xiao, Qiao Wang, Cikui Hu, Yixi Zhu, Feizhou Yin, Hongling Yi, Xiaoping Liu, Longfei J Kidney Cancer VHL Review Article Pheochromocytomas (PCCs) are rare neuroendocrine tumors that originate from chromaffin cells of the adrenal medulla, and paragangliomas (PGLs) are extra-adrenal pheochromocytomas. These can be mainly found in clinical syndromes including multiple endocrine neoplasia (MEN), von Hippel–Lindau (VHL) syndrome, neurofibromatosis-1 (NF-1) and familial paraganglioma (FPGL). PCCs and PGLs are thought to have the highest degree of heritability among human tumors, and it has been estimated that 60% of the patients have genetic abnormalities. This review provides an overview of the clinical syndrome and the genetic screening strategies of PCCs and PGLs. Comprehensive screening principles and strategies, along with specific screening based on clinical symptoms, biochemical tests and immunohistochemistry, are discussed. Codon Publications 2018-12-27 /pmc/articles/PMC6308242/ /pubmed/30613466 http://dx.doi.org/10.15586/jkcvhl.2018.113 Text en © Liu P et al. http://creativecommons.org/licenses/by/4.0 This open access article is licensed under Creative Commons Attribution 4.0 International (CC BY 4.0). |
spellingShingle | Review Article Liu, Peihua Li, Minghao Guan, Xiao Yu, Anze Xiao, Qiao Wang, Cikui Hu, Yixi Zhu, Feizhou Yin, Hongling Yi, Xiaoping Liu, Longfei Clinical Syndromes and Genetic Screening Strategies of Pheochromocytoma and Paraganglioma |
title | Clinical Syndromes and Genetic Screening Strategies of Pheochromocytoma and Paraganglioma |
title_full | Clinical Syndromes and Genetic Screening Strategies of Pheochromocytoma and Paraganglioma |
title_fullStr | Clinical Syndromes and Genetic Screening Strategies of Pheochromocytoma and Paraganglioma |
title_full_unstemmed | Clinical Syndromes and Genetic Screening Strategies of Pheochromocytoma and Paraganglioma |
title_short | Clinical Syndromes and Genetic Screening Strategies of Pheochromocytoma and Paraganglioma |
title_sort | clinical syndromes and genetic screening strategies of pheochromocytoma and paraganglioma |
topic | Review Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6308242/ https://www.ncbi.nlm.nih.gov/pubmed/30613466 http://dx.doi.org/10.15586/jkcvhl.2018.113 |
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