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Genetics of Pheochromocytomas and Paragangliomas Determine the Therapeutical Approach

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出版社

MDPI
DOI: 10.3390/ijms23031450

关键词

pheochromocytoma; paraganglioma; hereditary tumor; susceptibility genes; germline; somatic; metastatic; treatment; personalized medicine

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  1. National Laboratories Excellence program (under National Tumorbiology Laboratory project [NLP-17]

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Pheochromocytomas and paragangliomas are the most heritable endocrine tumors, most of which are benign and can be resolved through surgical removal. However, a small percentage are malignant with limited therapeutic options available.
Pheochromocytomas and paragangliomas are the most heritable endocrine tumors. In addition to the inherited mutation other driver mutations have also been identified in tumor tissues. All these genetic alterations are clustered in distinct groups which determine the pathomechanisms. Most of these tumors are benign and their surgical removal will resolve patient management. However, 5-15% of them are malignant and therapeutical possibilities for them are limited. This review provides a brief insight about the tumorigenesis associated with pheochromocytomas/paragangliomas in order to present them as potential therapeutical targets.

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