Pheochromocytoma/Paraganglioma: A Poster Child for Cancer Metabolism.

Pheochromocytoma/Paraganglioma: A Poster Child for Cancer Metabolism.

J Clin Endocrinol Metab. 2018 Feb 01;:

Authors: Tevosian SG, Ghayee HK

Abstract
Context: Pheochromocytomas (PCCs) are tumors that are derived from the chromaffin cells of the adrenal medulla. Extra-adrenal PCCs called paragangliomas (PGLs) are derived from the sympathetic and parasympathetic chain ganglia. PCCs secrete catecholamines which cause hypertension and have adverse cardiovascular consequences as a result of catecholamine excess. PGLs may or may not produce catecholamines depending on their genetic type and anatomical location. The most worrisome aspect of these tumors is their ability to become aggressive and metastasize, for which there are no known cures.
Methods: Original articles and reviews indexed in PubMed were identified by querying with specific PCC/PGL and Krebs Cycle pathway-related terms. Additional references were selected through the in depth analysis of the relevant publications.
Results: In this review, we primarily discussed Krebs Cycle mutations that can be instrumental in helping investigators to identify key biological pathways and molecules that may serve as biomarkers or treatment targets.
Conclusions: The mainstay of treatment for patients with PCC/PGLs is surgical. However, the tide may be turning with the discovery of new genes associated with PCC/PGLs that may shed light on oncometabolites utilized by these tumors.

PMID: 29409060 [PubMed - as supplied by publisher]

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