Cargando…

Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies

Pheochromocytoma and paraganglioma (PCPGs) are rare neuroendocrine tumors that arise from the chromaffin tissue of adrenal medulla and sympathetic ganglia. Although metastatic PCPGs account for only 10% of clinical cases, morbidity and mortality are high because of the uncontrollable mass effect and...

Descripción completa

Detalles Bibliográficos
Autores principales: Pang, Ying, Liu, Yang, Pacak, Karel, Yang, Chunzhang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6521122/
https://www.ncbi.nlm.nih.gov/pubmed/30925729
http://dx.doi.org/10.3390/cancers11040436
_version_ 1783418884014997504
author Pang, Ying
Liu, Yang
Pacak, Karel
Yang, Chunzhang
author_facet Pang, Ying
Liu, Yang
Pacak, Karel
Yang, Chunzhang
author_sort Pang, Ying
collection PubMed
description Pheochromocytoma and paraganglioma (PCPGs) are rare neuroendocrine tumors that arise from the chromaffin tissue of adrenal medulla and sympathetic ganglia. Although metastatic PCPGs account for only 10% of clinical cases, morbidity and mortality are high because of the uncontrollable mass effect and catecholamine level generated by these tumors. Despite our expanding knowledge of PCPG genetics, the clinical options to effectively suppress PCPG progression remain limited. Several recent translational studies revealed that PCPGs with different molecular subtypes exhibit distinctive oncogenic pathways and spectrum of therapy resistance. This suggests that therapeutics can be adjusted based on the signature molecular and metabolic pathways of PCPGs. In this review, we summarized the latest findings on PCPG genetics, novel therapeutic targets, and perspectives for future personalized medicine.
format Online
Article
Text
id pubmed-6521122
institution National Center for Biotechnology Information
language English
publishDate 2019
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-65211222019-05-31 Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies Pang, Ying Liu, Yang Pacak, Karel Yang, Chunzhang Cancers (Basel) Review Pheochromocytoma and paraganglioma (PCPGs) are rare neuroendocrine tumors that arise from the chromaffin tissue of adrenal medulla and sympathetic ganglia. Although metastatic PCPGs account for only 10% of clinical cases, morbidity and mortality are high because of the uncontrollable mass effect and catecholamine level generated by these tumors. Despite our expanding knowledge of PCPG genetics, the clinical options to effectively suppress PCPG progression remain limited. Several recent translational studies revealed that PCPGs with different molecular subtypes exhibit distinctive oncogenic pathways and spectrum of therapy resistance. This suggests that therapeutics can be adjusted based on the signature molecular and metabolic pathways of PCPGs. In this review, we summarized the latest findings on PCPG genetics, novel therapeutic targets, and perspectives for future personalized medicine. MDPI 2019-03-28 /pmc/articles/PMC6521122/ /pubmed/30925729 http://dx.doi.org/10.3390/cancers11040436 Text en © 2019 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Pang, Ying
Liu, Yang
Pacak, Karel
Yang, Chunzhang
Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies
title Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies
title_full Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies
title_fullStr Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies
title_full_unstemmed Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies
title_short Pheochromocytomas and Paragangliomas: From Genetic Diversity to Targeted Therapies
title_sort pheochromocytomas and paragangliomas: from genetic diversity to targeted therapies
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6521122/
https://www.ncbi.nlm.nih.gov/pubmed/30925729
http://dx.doi.org/10.3390/cancers11040436
work_keys_str_mv AT pangying pheochromocytomasandparagangliomasfromgeneticdiversitytotargetedtherapies
AT liuyang pheochromocytomasandparagangliomasfromgeneticdiversitytotargetedtherapies
AT pacakkarel pheochromocytomasandparagangliomasfromgeneticdiversitytotargetedtherapies
AT yangchunzhang pheochromocytomasandparagangliomasfromgeneticdiversitytotargetedtherapies