Epigenetics of pheochromocytoma and paraganglioma. (5th July 2018)

• Record Type: Journal Article
• Authors: Björklund, Peyman; Backman, Samuel
• Is Part Of: Molecular and cellular endocrinology. Volume 469(2018)
• Page Start: 92
• Page End: 97
• Publication Date: 2018-07-05
• Subjects: Pheochromocytoma -- Paraganglioma -- Epigenetics -- Adrenal -- Methylation; Endocrinology -- Periodicals; Molecular biology -- Periodicals; Cytology -- Periodicals; Endocrinology -- Periodicals; Hormones -- Periodicals; Endocrinologie -- Périodiques; Cytology; Endocrinology; Molecular biology; Periodicals; 573.4
• DOI: 10.1016/j.mce.2017.06.016
• Languages: English
• ISSNs: 0303-7207

Abstract

Abstract: Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors arising in the medullae of the adrenal glands or in paraganglia. The knowledge of the tumor biology of these lesions has increased dramatically during the past two decades and more than a dozen recurrently mutated genes have been identified. Different clusters have been described that share epigenetic signatures. Mutations in the succinate dehydrogenase complex subunit genes play a pivotal role in reprogramming the epigenetic state of these tumors by inhibiting epigenetic regulators such as TET enzymes and histone demethylases. Another subgroup of tumors carries hypomethylated genomes, and overexpression of several micro-RNAs has been described. While much remains to be investigated regarding the epigenetics of PPGLs, it is clear that it plays an important role in PPGL biology. Highlights: Pheochromocytomas and paragangliomas carry epigenetic aberrations. Succinate dehydrogenase mutations cause epigenetic dysregulation. Several genes involved in epigenetic modulation have been described mutated in pheochromocytomas and paragangliomas.