Pheochromocytoma/Paraganglioma: A Poster Child for Cancer Metabolism

Sergei G. Tevosian; Hans K. Ghayee


J Clin Endocrinol Metab. 2018;103(5):1779-1789. 

In This Article


Genetic analysis of affected patients and whole-genome approaches have substantially increased our understanding of PCC/PGL tumor biology. Although PCC/PGL tumors may have shown their many facets, like Dr. Jekyll and Mr. Hyde, commonalities that cause PCC/PGLs are now becoming apparent. Potential biomarkers such as 2HG, αKG, succinate, and IDH mutations are important candidates for identifying specific PCC/PGL phenotypes. The clinical and pharmacological applications of this research will continue to evolve, but it is already clear that genetic testing in all patients with PCC/PGL is required to identify the subtype of PCC/PGL and the patient's at-risk family members. New research in metabolic pathophysiology in PCC/PGL has played an important role in understanding the function of these tumors. With further advancement in translational research, targeted treatments can be developed on the basis of the pathways these tumors are using for their growth and survival. Understanding other important components, such as distinctive epigenetic modifications in tumor genomes and their effect on the metabolism, will be key for developing personalized therapy in the near future.