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Trends Endocrinol Metab. 2017 Nov;28(11):807-817. doi: 10.1016/j.tem.2017.08.001. Epub 2017 Aug 31.

New Insights into the Nuclear Imaging Phenotypes of Cluster 1 Pheochromocytoma and Paraganglioma.

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Department of Nuclear Medicine, La Timone University Hospital, European Center for Research in Medical Imaging (CERIMED), Aix-Marseille University, Marseille, France. Electronic address:
Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD, USA. Electronic address:


Pheochromocytomas and paragangliomas (PPGLs) belong to the family of neural crest cell-derived neoplasms. In up to 70% of cases they are associated with germline and somatic mutations in 15 well-characterized PPGL driver or fusion genes. PPGLs can be grouped into three main clusters, where cluster 1 includes PPGLs characterized by a pseudohypoxic signature. Although cluster 1 tumors share several common features, they exhibit unique behaviors. We present here unique insights into the imaging phenotypes of cluster 1 PPGLs based on glucose uptake, catecholamine metabolism, and somatostatin receptor expression. Recent data suggest that succinate is a major player in the imaging phenotype of succinate dehydrogenase-deficient PPGLs. This review emphasizes the emerging stromal cell-succinate interaction and highlights new perspectives in PPGL theranostics.


(68)Ga-DOTATATE; 6-[(18)F]-fluoro-L-3,4-dihydroxyphenylalanine; gallium radioisotopes; magnetic resonance; paraganglioma; pheochromocytoma; positron emission tomography; radionuclide therapy; somatostatin; theranostics

[Available on 2018-11-01]
[Indexed for MEDLINE]

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